Genetic, metabolic, and biochemical aspects of the porphyrias.

The human porphyrias comprise a group of inherited and acquired disorders characterized by aberrations in activities of specific enzymes of the heme biosynthetic pathway. The nosology, natural history, and clinical characteristics of these diseases have been well reviewed by others (Taddeini and Watson, 1968; Dean, 1971; Tschudy and Lamon, 1980; Meyer and Schmid, 1978) and will be described in this review only to the extent that the biochemistry and genetics of these disorders seem to bear on their clinical presentations.

[1]  B. Shanley,et al.  Decreased uroporphyrinogen decarboxylase activity in 'experimental symptomatic porphyria'. , 1971, Life sciences. Pt. 2: Biochemistry, general and molecular biology.

[2]  C. Petitclerc,et al.  [Congenital erythropoietic porphyria]. , 1972, Canadian Medical Association journal.

[3]  S. Granick,et al.  Control of hemoglobin synthesis in the cultured chick blastoderm. , 1967, The Journal of biological chemistry.

[4]  G. Abbritti,et al.  Decreased liver activity of porphyrin-metal chelatase in hepatic porphyria caused by 3,5-diethoxycarbonyl-1,4-dihydrocollidine. Studies in rats and mice. , 1973, The Biochemical journal.

[5]  D. Bickers,et al.  Porphyria Cutanea Tarda in Two Patients Treated with Hemodialysis for Chronic Renal Failure , 1978 .

[6]  H. Yoshikawa,et al.  Incorporation of iron in the haem moiety of chromoproteins , 1964 .

[7]  P. Anderson,et al.  Purification and properties of uroporphyrinogen I synthase from human erythrocytes. Identification of stable enzyme-substrate intermediates. , 1980, The Journal of biological chemistry.

[8]  J. Neilands,et al.  Zinc, an essential metal ion for beef liver δ-aminolevulinate dehydratase , 1973 .

[9]  P. Sneath,et al.  Studies in congenital porphyria. 2. Incorporation of 15N in the stercobilin in the normal and in the porphyric. , 1950, The Biochemical journal.

[10]  P. Marks,et al.  Differentiation of normal and neoplastic hematopoietic cells , 1978 .

[11]  B. Leijnse,et al.  Acquired porphyria from a liver tumour. , 1957, Clinical science.

[12]  C. J. Watson.  Hematin and Porphyria , 1975 .

[13]  D. Handley,et al.  Erythropoietic uroporphyria of Gunther first presenting at 58 years with positive family studies. , 1975, British medical journal.

[14]  S. Sassa,et al.  Studies in porphyria. IV. Expression of the gene defect of acute intermittent porphyria in cultured human skin fibroblasts and amniotic cells: prenatal diagnosis of the porphyric trait , 1975, The Journal of experimental medicine.

[15]  J. Paterniti,et al.  delta-Aminolevulinic acid synthetase from rat liver mitochondria. Purification and properties. , 1979, The Journal of biological chemistry.

[16]  L. Soyka,et al.  Further studies on the inhibition of drug metabolism by pregnanolone and related steroids. , 1974, Biochemical pharmacology.

[17]  S. Granick,et al.  Induction of -aminolevulinic acid synthetase in chick embryo liver cells in cluture. , 1970, Proceedings of the National Academy of Sciences of the United States of America.

[18]  C. Pierach,et al.  Neurotoxicity of δ-aminolevulinic acid and porphobilinogen , 1978, Experimental Neurology.

[19]  N. Hayashi,et al.  Translocation of delta-aminolevulinate synthase from the cytosol to the mitochondria and its regulation by hemin in the rat liver. , 1980, The Journal of biological chemistry.

[20]  J. Goldstein,et al.  Effects of pentachlorophenol on hepatic drug-metabolizing enzymes and porphyria related to contamination with chlorinated dibenzo-p-dioxins and dibenzofurans. , 1977, Biochemical pharmacology.

[21]  R. Estabrook,et al.  The interaction of steroids with liver microsomal cytochrome P-450--a general hypothesis. , 1975, Journal of steroid biochemistry.

[22]  M. Mathews-Roth,et al.  Erythropoietic protoporphyria. 10 years experience. , 1976, The American journal of medicine.

[23]  H. Ippen.  [General symptoms of late skin porphyria (porphyria cutanea tarda) as an indication for its treatment]. , 1961, Deutsche medizinische Wochenschrift.

[24]  Z. Petryka,et al.  Repression by hematin of porphyrin biosynthesis in erythrocyte precursors in congenital erythropoietic porphyria. , 1974, Proceedings of the National Academy of Sciences of the United States of America.

[25]  J. Paterniti,et al.  Detection and regulation of δ-aminolevulinic acid synthetase activity in the rat brain , 1978 .

[26]  J. Seehra,et al.  Mechanism of action of 5-aminolevulinic acid dehydratese: stepwise order of addition of the two molecules of 5-aminolevulinic acid in the enzymic synthesis of porphobilinogen , 1980 .

[27]  J. S. Taylor,et al.  Porphyria cutanea tarda in three generations of a single family. , 1978, The New England journal of medicine.

[28]  W. J. Turner.  STUDIES ON PORPHYRIA I. OBSERVATIONS ON THE FOX-SQUIRREL, SCIURUS NIGER , 1937 .

[29]  J. S. Woods,et al.  Perinatal differences in delta-aminolevulinic acid synthetase activity. , 1970, Life sciences. Pt. 2: Biochemistry, general and molecular biology.

[30]  T. Tephly,et al.  Cardiac delta-aminolevulinic acid synthetase activity. Effects of fasting, cobaltous chloride and hemin. , 1980, Biochemical pharmacology.

[31]  È. Derrien.  Sur l'accumulation d'une porphyrine dans la glande de Harder des rongeurs du genre Mus et sur son mode d'excretion. , 1924 .

[32]  J. Kushner,et al.  Hepatic siderosis and porphyria cutanea tarda: relation of iron excess to the metabolic defect. , 1977, Seminars in hematology.

[33]  S. Granick,et al.  Control of hemoglobin synthesis in the cultured chick blastoderm by delta-aminolevulinic acid synthetase: increase in the rate of hemoglobin formation with delta-aminolevulinic acid. , 1965, Proceedings of the National Academy of Sciences of the United States of America.

[34]  C. Magnussen,et al.  Acute intermittent porphyria: clinical and selected research aspects. , 1975, Annals of internal medicine.

[35]  H. Haberman,et al.  Porphyria cutanea tarda: comparison of cases precipitated by alcohol and estrogens. , 1975, Canadian Medical Association journal.

[36]  G. Kikuchi,et al.  Mechanism of allylisopropylacetamide-induced increase of -aminolevulinate synthetase in liver mitochondria. VI. Multiple molecular forms of -aminolevulinate synthetase in the cytosol and mitochondria of induced cock liver. , 1972, Archives of biochemistry and biophysics.

[37]  J. Bloomer,et al.  Heme synthetase deficiency in human protoporphyria. Demonstration of the defect in liver and cultured skin fibroblasts. , 1975, The Journal of clinical investigation.

[38]  H. L. Mason,et al.  Adult form of chronic porphyria with cutaneous manifestations; report of seventeen additional cases. , 1951, Journal of the American Medical Association.

[39]  W. Polglase,et al.  Aerobic and anaerobic coproporphyrinogenase activities in extracts from Saccharomyces cerevisiae. , 1974, The Journal of biological chemistry.

[40]  J. S. Woods,et al.  Studies of the perinatal differences in the activity of hepatic -aminolevulinic acid synthetase. , 1972, Biochemical pharmacology.

[41]  J. Strik.  Toxicity of PBBs with special reference to porphyrinogenic action and spectral interaction with hepatic cytochrome P-450. , 1978, Environmental health perspectives.

[42]  R. Aldrich,et al.  An isotopic study of porphyrin and hemoglobin metabolism in a case of porphyria. , 1949, The Journal of biological chemistry.

[43]  R. Schmid,et al.  The sources of bile pigment in the rat: studies of the "early labeled" fraction. , 1966, The Journal of clinical investigation.

[44]  G. Drummond,et al.  Manganese and zinc blockade of enzyme induction: studies with microsomal heme oxygenase. , 1979, Proceedings of the National Academy of Sciences of the United States of America.

[45]  N. Mcintyre,et al.  HEPATIC δ-AMINOLÆVULINIC ACID SYNTHETASE IN AN ATTACK OF HEREDITARY COPROPORPHYRIA AND DURING REMISSION , 1971 .

[46]  R. Frydman,et al.  Studies on porphobilinogen deaminase and uroporphyrinogen 3 cosynthase from human erythrocytes. , 1974, Biochimica et biophysica acta.

[47]  J. Kushner,et al.  The Role of Iron in the Pathogenesis of Porphyria Cutanea Tarda , 2013 .

[48]  G. Patton,et al.  Studies on hepatic -aminolevulinic acid synthetase. , 1973, The Journal of biological chemistry.

[49]  T. Fitzpatrick,et al.  Year Book of Dermatology , 1971, Journal of the Royal College of Physicians of London.

[50]  C. Rimington,et al.  Symptomatic porphyria in a case of Felty's syndrome. I. Clinical and routine biochemcial studies. , 1972, Clinical chemistry.

[51]  L. Varticovski,et al.  Biosynthesis of porphyrin precursors. Purification and characterization of mammalian L-alanine:gamma,delta-dioxovaleric acid aminotransferase. , 1980, The Journal of biological chemistry.

[52]  D. Bevan,et al.  Mechanism of porphobilinogen synthase. Requirement of Zn2+ for enzyme activity. , 1980, The Journal of biological chemistry.

[53]  P. Tofilon,et al.  Measurement and regulation of rat testicular δ-aminolevulinic acid synthetase activity , 1980 .

[54]  S. Granick,et al.  Steroid induction of porphyrin synthesis in liver cell culture. II. The effects of heme, uridine diphosphate glucuronic acid, and inhibitors of nucleic acid and protein synthesis on the induction process. , 1968, The Journal of biological chemistry.

[55]  Z. Petryka,et al.  Porphyria variegata and porphyria cutanea tarda in siblings: chemical and genetic aspects. , 1975, Proceedings of the National Academy of Sciences of the United States of America.

[56]  J. Bornstein,et al.  Inhibition of the evoked release of acetylcholine by the porphyrin precursor δ‐aminolevulinic acid , 1979 .

[57]  C. J. Watson,et al.  Acute intermittent porphyria. Urinary porphobilinogen and other Ehrlich reactors in diagnosis. , 1961, JAMA.

[58]  S. Granick,et al.  CHAPTER 4 – δ-Aminolevulinic Acid Synthetase and the Control of Heme and Chlorophyll Synthesis*† , 1971 .

[59]  W. H. Elliott,et al.  Synthesis of delta-aminolaevulinate synthase in vitro using hepatic mRNA from chick embryos with induced porphyria. , 2005, European journal of biochemistry.

[60]  T. Masuya.  Pathophysiological observations on porphyrias. , 1969, Nihon Ketsueki Gakkai zasshi : journal of Japan Haematological Society.

[61]  R. Joseph,et al.  DEMONSTRATION OF THE DEFECT IN LIVER AND CULTURED SKIN FIBROBLASTS , 1975 .

[62]  Klock Jc,et al.  Functional changes in neutrophils collected by filtration leukapheresis and their relationship to cellular events that occur during adherence of neutrophils to nylon fibers. , 1979 .

[63]  H. H. Hoyt,et al.  Some studies of the comparative biology of human and bovine erythropoietic porphyria. , 1959, A.M.A. archives of internal medicine.

[64]  V. N. Finelli,et al.  Interaction of zinc and lead on δ-aminolevulinate dehydratase , 1975 .

[65]  U. Meyer.  Intermittent Acute Porphyria , 1973 .

[66]  G. Romeo,et al.  UROPORPHYRINOGEN III COSYNTHETASE IN HUMAN CONGENITAL ERYTHROPOIETIC PORPHYRIA , 1969 .

[67]  C. Rimington,et al.  Porphyrin studies in congenital erythropoietic porphyria. Studies on a sample of faeces and a sample of urine from a patient with C.E.P. and comparison with a bovine case of this disease. , 1973, Danish medical bulletin.

[68]  R. Schmid.  Cutaneous porphyria in Turkey. , 1960, The New England journal of medicine.

[69]  D. Coleman.  Purification and properties of delta-aminolevulinate dehydratase from tissues of two strains of mice. , 1966, The Journal of biological chemistry.

[70]  F. DeMatteis.  Drug interactions in experimental hepatic porphyria. A model for the exacerbation by drugs of human variegate porphyria. , 1973 .

[71]  R. Freshney,et al.  The activities of three enzymes of haem synthesis during hepatic erythropoiesis in the mouse embryo. , 1971, Journal of embryology and experimental morphology.

[72]  D. Bissell,et al.  Cytochrome P-450 heme and the regulation of δ-aminolevulinic acid synthetase in the liver , 1976 .

[73]  H. Moses,et al.  THE INFLUENCE OF POSTNATAL DEVELOPMENT ON DRUG-INDUCED HEPATIC PORPHYRIA AND THE SYNTHESIS OF CYTOCHROME P-450 , 1971, The Journal of experimental medicine.

[74]  S. Granick,et al.  Biosynthesis of delta-aminolevulinic acid from the intact carbon skeleton of glutamic acid in greening barley. , 1975, Proceedings of the National Academy of Sciences of the United States of America.

[75]  K. Jones,et al.  Porphyria cutanea tarda: clinical and laboratory features. , 1979, Canadian Medical Association journal.

[76]  B. Webber,et al.  Hepatic haem metabolism in porphyria cutanea tarda (PCT): enzymatic studies and their relation to liver ultrastructure. , 1976, Annals of clinical research.

[77]  G. Rovera.  [Steroids and hematopoiesis]. , 1969, Recenti progressi in medicina.

[78]  T. With,et al.  PORPHYRIA IN DOMESTIC ANIMALS: DANISH OBSERVATIONS IN PIGS AND CATTLE AND COMPARISON WITH HUMAN PORPHYRIA , 1963, Annals of the New York Academy of Sciences.

[79]  J. Hutton,et al.  Chemical induction of hepatic porphyria in inbred strains of mice. , 1970, Archives of biochemistry and biophysics.

[80]  G. Korting.  Über Porphyria-cutanea-tarda-artige Hautveränderungen bei Langzeithämodialysepatienten , 1975 .

[81]  G. Elder,et al.  Uroporphyrinogen decarboxylase activity of human tissues [proceedings]. , 1977, Biochemical Society transactions.

[82]  C. J. Watson,et al.  Some studies of the nature and clinical significance of porphobilinogen. , 1954, Transactions of the Association of American Physicians.

[83]  A. M. del Batlle,et al.  Studies on porphyrin biosynthesis and enzymes involved in bovine congenital erythropoietic porphyria. , 1979, Clinical science.

[84]  L. Eales,et al.  THE HISTOPATHOLOGY OF THE LIVER IN ACQUIRED (SYMPTOMATIC) PORPHYRIA. , 1963, South African journal of laboratory and clinical medicine. Suid-Afrikaanse tydskrif vir laboratorium- en kliniekwerk.

[85]  G. Padmanaban,et al.  A model for the regulation of δ-aminolaevulinate synthetase induction in rat liver , 1973 .

[86]  H. Marver,et al.  Microsomal heme oxygenase. Characterization of the enzyme. , 1969, The Journal of biological chemistry.

[87]  M. Moore,et al.  Excessive urinary excretion of certain porphyrinogenic steroids in human acute intermittent porphyria. , 1969, Lancet.

[88]  Warin Rp.  PORPHYRIA CUTANEA TARDA ASSOCIATED WITH OESTROGEN THERAPY FOR PROSTATIC CARCINOMA. , 1963, The British journal of dermatology.

[89]  F. De Matteis,et al.  Inhibition of protohaem ferro-lyase in experimental porphyria. Isolation and partial characterization of a modified porphyrin inhibitor. , 1980, The Biochemical journal.

[90]  B. Grandchamp,et al.  Decreased lymphocyte coproporphyrinogen III oxidase activity in hereditary coproporphyria. , 1977, Biochemical and biophysical research communications.

[91]  C. Pierach,et al.  Use of hematin in the acute attack of the "inducible" hepatic prophyrias. , 1978, Advances in internal medicine.

[92]  G. Y. Kennedy.  PORPHYRINS IN INVERTEBRATES , 1975, Annals of the New York Academy of Sciences.

[93]  A. Redeker,et al.  ERYTHROPOIETIC PROTOPORPHYRIA PRESENTING AS HYDROA AESTIVALE. , 1964, Archives of dermatology.

[94]  A. Waxman,et al.  Oscillations of hepatic δ-aminolevulinic acid synthetase produced in, vivo by heme , 1966 .

[95]  R. Poulson.  The enzymic conversion of protoporphyrinogen IX to protoporphyrin IX in mammalian mitochondria. , 1976, The Journal of biological chemistry.

[96]  J. Bloomer.  Characterization of deficient heme synthase activity in protoporphyria with cultured skin fibroblasts. , 1980, The Journal of clinical investigation.

[97]  S. Nacht,et al.  Human porphyria cutanea tarda. Isolation and properties of the urinary porphyrins. , 1970, Clinica chimica acta; international journal of clinical chemistry.

[98]  L. Eales,et al.  PORPHYRIA-THE ACUTE ATTACK , 1962 .

[99]  C. Rimington,et al.  Complete porphyrin analysis of pathological urines. , 1949, Scandinavian journal of clinical and laboratory investigation.

[100]  A. Kappas,et al.  Studies on the mechanism of induction of haem oxygenase by cobalt and other metal ions. , 1976, The Biochemical journal.

[101]  S. Snyder,et al.  δ‐Aminolevulinic acid: Influences on synaptic GABA receptor binding may explain CNS symptoms of porphyria , 1977, Annals of neurology.

[102]  G. Blekkenhorst,et al.  Enzymatic Defects in Hepatic Porphyria , 1973 .

[103]  A. Goldstein,et al.  Purification and properties , 1975 .

[104]  M. Doss.  Porphyrins in human diseases , 1976 .

[105]  C. Rimington,et al.  Purification and properties of coproporphyrinogenase. , 1965, The Biochemical journal.

[106]  T. Tephly,et al.  Studies on adrenal δ-aminolevulinic acid synthetase , 1976 .

[107]  N. Jagani,et al.  548 PRENATAL DIAGNOSIS OF CONGENITAL ERYTHROPOIETIC ETIC PORPHYRIA , 1978, Pediatric Research.

[108]  P. Lynch,et al.  Erythropoietic protoporphyria. Report of a family and a clinical review. , 1965, Archives of dermatology.

[109]  S. Sassa,et al.  The influence of steroid hormone metabolites on the in vitro development of erythroid colonies derived from human bone marrow , 1979, The Journal of experimental medicine.

[110]  K. Jones,et al.  Association between induction of aryl hydrocarbon hydroxylase and depression of uroporphyrinogen decarboxylase activity. , 1977, Research communications in chemical pathology and pharmacology.

[111]  J. Pritchard,et al.  IRON STORES OF NORMAL ADULTS AND REPLENISHMENT WITH ORAL IRON THERAPY. , 1964, JAMA.

[112]  S. Lindstedt,et al.  Excretion of δ-aminolevulinic acid in hereditary tyrosinemia , 1969 .

[113]  S. Sassa,et al.  Sequential induction of heme pathway enzymes during erythroid differentiation of mouse Friend leukemia virus-infected cells , 1976, The Journal of experimental medicine.

[114]  I. Magnus,et al.  Iodine tungsten fluorescence microscopy for porphyrin fluorescence. A study on erythropoietic protoporphyria. , 1966, Archives of dermatology.

[115]  L. Eales,et al.  Porphyria--the acute attack. An analysis of 80 cases. , 1962, South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde.

[116]  S. Sassa,et al.  Induction of aminolevulinate synthase and porphyrins in cultured liver cells maintained in chemically defined medium. Permissive effects of hormones on induction process. , 1977, The Journal of biological chemistry.

[117]  E. Wilson,et al.  Beef‐Liver 5‐Aminolevulinic Acid Dehydratase , 1972 .

[118]  G. Elder,et al.  Decreased activity of hepatic uroporphyrinogen decarboxylase in sporadic porphyria cutanea tarda. , 1978, The New England journal of medicine.

[119]  A. Castells,et al.  A CASE OF BIOCHEMICALLY UNCLASSIFIABLE HEPATIC PORPHYRIA , 1969, The British journal of dermatology.

[120]  N. Whittaker,et al.  Hereditary coproporphyria with acute intermittent manifestations. , 1965, British medical journal.

[121]  G. Dean.  The Porphyrias: A Story of Inheritance and Environment , 1964 .

[122]  T. Tephly,et al.  Induction of hepatic mitochondrial ferrochelatase by phenobarbital. , 1970, Biochemical and biophysical research communications.

[123]  L. Eriksen,et al.  Porphyrin distribution and porphyrin excretion in human congenital erythropoietic porphyria. , 1974, Scandinavian journal of clinical and laboratory investigation.

[124]  P. Mustajoki,et al.  Hereditary hepatic porphyrias in Finland. , 2009, Acta medica Scandinavica.

[125]  R. I. Birchfield,et al.  Acute intermittent porphyria with seizures. Anticonvulsant medication-induced metabolic changes. , 1966, American journal of diseases of children.

[126]  S. Lindstedt,et al.  Dietary treatment in tyrosinemia (tyrosinosis). With a note on the possible recognition of the carrier state. , 1967, American journal of diseases of children.

[127]  H. Marver,et al.  Biochemical defects in two types of human hepatic porphyria. , 1970, The New England journal of medicine.

[128]  L. Eales,et al.  THE CLINICAL BIOCHEMISTRY OF THE HUMAN HEPATOCUTANEOUS PORPHYRIAS IN THE LIGHT OF RECENT STUDIES OF NEWLY IDENTIFIED INTERMEDIATES AND PORPHYRIN DERIVATIVES * , 1975, Annals of the New York Academy of Sciences.

[129]  B. Yoda,et al.  Mechanism of allylisopropylacetamide-induced increase of delta-aminolevulinate synthetase in liver mitochondria. II. Effects of hemin and bilirubin on enzyme induction. , 1968, Journal of biochemistry.

[130]  A. Goeij,et al.  Decreased Haem Synthetase Activity in Blood Cells of Patients with Erythropoietic Protoporphyria , 1975, European journal of clinical investigation.

[131]  D. Gemsa.  MICROSOMAL HEME OXYGENASE , 1981 .

[132]  S. Granick,et al.  Studies in lead poisoning. II. Correlation between the ratio of activated to inactivated delta-aminolevulinic acid dehydratase of whole blood and the blood lead level. , 1973, Biochemical medicine.

[133]  C. J. Stevenson,et al.  PHOTOTOXIC BULLOUS ERUPTIONS DUE TO NALIDIXIC ACID , 1969, British Journal of Dermatology.

[134]  J. Waldenström,et al.  Different Patterns of Human Porphyria* , 1963, British medical journal.

[135]  S. Granick,et al.  Delta-Aminolevulinic acid synthase from chick embryo liver mitochondria. I. Purification and some properties. , 1976, The Journal of biological chemistry.

[136]  K. Kluge,et al.  [COMBINED HEREDITARY PORPHYRIA HEPATICA (HEREDITARY COPROPORPHYRIA)]. , 1965, Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete.

[137]  G. Krook,et al.  Erythropoietic protoporphyria. A study of known cases in Sweden. , 2009, Acta medica Scandinavica. Supplementum.

[138]  J. Epstein,et al.  PORPHYRIA CUTANEA TARDA SYMPTOMATICA (PCT-S): A STUDY OF THE EFFECT OF PHLEBOTOMY THERAPY. , 1965, Archives of dermatology.

[139]  N. Katunuma,et al.  A new protease inactivating delta-aminolevulinic acid synthetase in mitochondria of human bone marrow cells. , 1975, Biochemical and biophysical research communications.

[140]  M. Moore,et al.  Amounts of faecal porphyrin-peptide conjugates in the porpyrias. , 1972, Clinical science.

[141]  L. Taddeini,et al.  A SUGGESTED CONTROL GENE MECHANISM FOR THE EXCESSIVE PRODUCTION OF TYPES I AND 3 PORPHYRINS IN CONGENITAL ERYTHROPOIETIC PORPHYRIA. , 1964, Proceedings of the National Academy of Sciences of the United States of America.

[142]  D. Shurtleff,et al.  Congenital erythropoietic porphyria. I. Case report, special studies and therapy. , 1968, The American journal of medicine.

[143]  L. Jirásek,et al.  [Chloracne, porphyria cutanea tarda, and other poisonings due to the herbicides]. , 1976, Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete.

[144]  R. Labbé,et al.  Modified erythrocyte uroporphyrinogen I synthase assay, and its clinical interpretation. , 1978, Clinical chemistry.

[145]  B. Grandchamp,et al.  HOMOZYGOUS CASE OF HEREDITARY COPROPORPHYRIA , 1977, The Lancet.

[146]  J. Kushner,et al.  The role of iron in the pathogenesis of porphyria cutanea tarda. An in vitro model. , 1972, The Journal of clinical investigation.

[147]  G. Kikuchi,et al.  Induction of delta-aminolevulinate synthetase in organ culture of chick embryo liver by allylisopropylacetamide and 3,5-dicarbethoxy-1,4-dihydrocollidine. , 1974, Journal of biochemistry.

[148]  E. Dowdle,et al.  The diagnostic importance of faecal porphyrins in the differentiation of the porphyrias. 3. South African genetic porphyria (variegcte porphyria)--the acute attack. , 1966, South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde.

[149]  P. Leder,et al.  Globin messenger-RNA induction during erythroid differentiation of cultured leukemia cells. , 1972, Proceedings of the National Academy of Sciences of the United States of America.

[150]  G. Padgett,et al.  Feline congenital erythropoietic porphyria associated with severe anemia and renal disease. Clinical, morphologic, and biochemical studies. , 1975, The American journal of pathology.

[151]  S. Granick.  The structural and functional relationships between heme and chlorophyll. , 1948, Harvey lectures.

[152]  S. Robinson,et al.  Early-labelled haem in erythroid and hepatic cells , 1975, Nature.

[153]  B. Felsher,et al.  Red-cell uroporphyrinogen decarboxylase activity in porphyria cutanea tarda and in other forms of porphyria. , 1978, The New England journal of medicine.

[154]  C. Fookes,et al.  Order of assembly of the four pyrrole rings during biosynthesis of the natural porphyrins , 1979 .

[155]  V. Flyger,et al.  Erythropoietic porphyria of the fox squirrel Sciurus niger. , 1973, The Journal of clinical investigation.

[156]  F. Takaku,et al.  Different responses of the hepatic and erythropoietic σ-aminolevulinic acid synthetase of mice , 1967 .

[157]  E. Donaldson,et al.  A Family Study. , 1962 .

[158]  S. Granick,et al.  ENZYMES OF PORPHYRIN SYNTHESIS IN RED BLOOD CELLS , 1958, Annals of the New York Academy of Sciences.

[159]  V. N. Finelli,et al.  Tyrosinemia with acute intermittent porphyria: aminolevulinic acid dehydratase deficiency related to elevated urinary aminolevulinic acid levels. , 1977, The Journal of pediatrics.

[160]  K. Keczkes,et al.  Bullous dermatosis of chronic renal failure , 1976, The British journal of dermatology.

[161]  D. Bickers,et al.  Induction of a deficiency of steroid delta 4-5 alpha-reductase activity in liver by a porphyrinogenic drug. , 1977, The Journal of clinical investigation.

[162]  B. Grandchamp,et al.  Some kinetic properties of human red cell uroporphyrinogen decarboxylase. , 1980, Biochimica et biophysica acta.

[163]  A. Goldberg.  Acute intermittent porphyria: a study of 50 cases. , 1959, The Quarterly journal of medicine.

[164]  S. Gross.  HEMATOLOGIC STUDIES ON ERYTHROPOIETIC PORPHYRIA: A NEW CASE WITH SEVERE HEMOLYSIS, CHRONIC THROMBOCYTOPENIA, AND FOLIC ACID DEFICIENCY. , 1964, Blood.

[165]  D. Tschudy,et al.  The effect of carbohydrate feeding on the induction of δ-aminolevulinic acid synthetase , 1964 .

[166]  W. Whetsell,et al.  Studies on porphyrin-heme biosynthesis in organotypic cultures of chick dorsal root ganglia. II. The effect of lead. , 1979, Environmental research.

[167]  M. Mihm,et al.  Bullous dermatosis of hemodialysis. , 1975, Annals of internal medicine.

[168]  R. Waddington.  A case of primary liver tumour associated with porphyria , 1972, The British journal of surgery.

[169]  L. Bogorad,et al.  δ-Aminolevulinic Acid Transaminase in Chlorella vulgaris , 1968 .

[170]  Delta-Aminolevulinic acid synthetase in the heart. , 1976, The Journal of biological chemistry.

[171]  M. Freedman,et al.  Hemin feedback inhibition at reticulocyte δ-aminolevulinic acid synthetase and δ-aminolevulinic acid dehydratase , 1978 .

[172]  M. Carstens,et al.  Effects of hexachlorobenzene feeding and iron overload on enzymes of haem biosynthesis and cytochrome P 450 in rat liver. , 1977, Clinical science and molecular medicine.

[173]  M. Moore,et al.  The biosynthesis of haem in congenital (erythropoietic) porphyria. , 1978, The International journal of biochemistry.

[174]  Z. Petryka,et al.  The activities of uroporphyrinogen synthetase and cosynthetase in congenital erythropoietic porphyria (cep) , 1976, American journal of hematology.

[175]  S. Granick,et al.  Increase in activity of alpha-aminolevulinic acid synthetase in liver mitochondria induced by feeding of 3,5-dicarbethoxy-1,4-dihydrocollidine. , 1963, The Journal of biological chemistry.

[176]  J. Zingraff,et al.  Porphyria cutanea tarda-like syndrome in hemodialyzed patients. , 1978, Nephron.

[177]  Cripps Dj,et al.  Hepatic and erythropoietic protoporphyria. Delta-aminolevulinic acid synthetase, fluorescence, and microfluorospectrophotometric study. , 1971 .

[178]  M. Whiting.  Synthesis of delta-aminolaevulinate synthase by isolated liver polyribosomes. , 1976, The Biochemical journal.

[179]  D. Brenner,et al.  The Enzymatic Defect in Variegate Porphyria , 1980 .

[180]  S. Granick,et al.  The induction in vitro of the synthesis of delta-aminolevulinic acid synthetase in chemical porphyria: a response to certain drugs, sex hormones, and foreign chemicals. , 1966, The Journal of biological chemistry.

[181]  M. Pathak,et al.  Acute intermittent porphyria. Increased ALA-synthetase activity during an acute attack. , 1970, Brain : a journal of neurology.

[182]  M. Moore,et al.  Heme biosynthesis in Friend erythroleukemia cells: control by ferrochelatase. , 1979, Proceedings of the National Academy of Sciences of the United States of America.

[183]  M. Kikutani,et al.  Harderian gland. V. Effect of dietary pantothemic acid deficiency on porphyrin biosynthesis in Harderian gland of rats. , 1975, Chemical and pharmaceutical bulletin.

[184]  P. Spooner,et al.  The regulation of haemoglobin synthesis in cultured chick blastoderms by steroids related to 5beta-androstane. , 1976, The Biochemical journal.

[185]  L. Rosenberg,et al.  Metabolic control and disease , 1980 .

[186]  S. Matlin,et al.  Macrocyclic intermediates in the biosynthesis of porphyrins. , 1976, Philosophical transactions of the Royal Society of London. Series B, Biological sciences.

[187]  H. Merker,et al.  Porphyria erythropoetica congenita Günther , 1963 .

[188]  D. Becker,et al.  Reduced Ferrochelatase Activity: a Defect Common to Porphyria Variegata and Protoporphyria , 1977, British journal of haematology.

[189]  W. B. Peirano,et al.  δ-Aminolevulinate dehydratase, a zinc dependent enzyme , 1974 .

[190]  D. Dolphin,et al.  Copper coproporphyrin excretion in familial coproporphyria. , 1978, Clinical chemistry.

[191]  J. A. Johnson,et al.  Erythropoietic defects in protoporphyria: a study of factors involved in labelling of porphyrins and bile pigments from ALA- 3 H and glycine- 14 C. , 1971, The Journal of laboratory and clinical medicine.

[192]  V. Flyger,et al.  Animal model: normal porphyria of fox squirrels (Sciurus niger). , 1977, The American journal of pathology.

[193]  J Waldenström,et al.  The porphyrias: a genetic problem. , 1967, Progress in medical genetics.

[194]  Longmore Db.  ELECTRICAL STIMULATION OF THE BLADDER: A NEW PROSPECT AFTER SPINAL INJURY? , 1965 .

[195]  T. Tephly.  Inhibition of Liver Hemoprotein Synthesis , 1978 .

[196]  G. Lucier,et al.  Toxicological assessment of hexachlorobiphenyl isomers and 2,3,7,8,-tetrachlorodibenzofuran in chicks. II. Effects on drug metabolism and porphyrin accumulation. , 1976, Toxicology and applied pharmacology.

[197]  G. Ruth,et al.  Bovine protoporphyria: the first nonhuman model of this hereditary photosensitizing disease. , 1977, Science.

[198]  G. Sweeney.  PATTERNS OF PORPHYRIN EXCRETION IN SOUTH AFRICA PORPHYRIC PATIENTS. , 1963, South African journal of laboratory and clinical medicine. Suid-Afrikaanse tydskrif vir laboratorium- en kliniekwerk.

[199]  F. Takaku,et al.  Purification and some properties of δ-aminolevulinate(ALA) synthetase in rabbit reticulocytes , 1971 .

[200]  Carlson,et al.  HEREDITARY COPROPORPHYRIA: DEMONSTRATION OF THE ABNORMALITIES IN HAEM BIOSYNTHESIS IN PERIPHERAL BLOOD , 2013 .

[201]  R. Lockshin,et al.  Stimulation of cytochrome c synthesis in the developing Polyphemus moth by -aminolevulinic acid. , 1971, Biochemistry.

[202]  R. Kato.  Effect of administration of 3-aminotriazole on the activity of microsomal drug-metabolizing enzyme systems of rat liver. , 1967, Japanese journal of pharmacology.

[203]  A. Kappas,et al.  Metals as regulators of heme metabolism. , 1977, Science.

[204]  R. Schmid,et al.  Porphyrin content of bone marrow and liver in the various forms of porphyria. , 1954, A.M.A. archives of internal medicine.

[205]  W. Polglase,et al.  The enzymic conversion of protoporphyrinogen IX to protoporphyrin IX. Protoporphyrinogen oxidase activity in mitochondrial extracts of Saccharomyces cerevisiae. , 1975, The Journal of biological chemistry.

[206]  A. Kappas,et al.  Inhibited induction of hepatic δ‐aminolevulinate synthetase in pregnancy , 1974 .

[207]  J. Price.  A neuropsychiatric and genetical investigation of acute intermittent porphyria ?: Svenska Bokfrlaget Norstedts, Stockholm, (pp. 87. Price not stated). , 1968 .

[208]  C. Pierach,et al.  Postulated deficiency of hepatic heme and repair by hematin infusions in the "inducible" hepatic porphyrias. , 1977, Proceedings of the National Academy of Sciences of the United States of America.

[209]  J. Zinkl,et al.  Erythrocytic enzymes and glycolytic intermediates in the normal bovine and in bovine erythropoietic porphyria. , 1973, Comparative biochemistry and physiology. A, Comparative physiology.

[210]  R. J. Porra,et al.  The enzymic conversion of coproporphyrinogen III into protoporphyrin IX , 1964 .

[211]  G. Elder,et al.  Faecal 'X porphyrin' in the hepatic porphyrias. , 1974, Enzyme.

[212]  L. Eales,et al.  The porphyrin peptides: practical implications. , 1971, South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde.

[213]  C. J. Watson,et al.  The serum porphobilinogen and hepatic porphobilinogen deaminase in normal and porphyric individuals. , 1971, The Journal of laboratory and clinical medicine.

[214]  R. J. Porra,et al.  Ferrochelatase and -aminolaevulate synthetase in brain, heart, kidney and liver of normal and porphyric rats. The induction of -aminolaevulate synthetase in kidney cytosol and mitochondria by allylisopropylacetamide. , 1971, The Biochemical journal.

[215]  S. Lindstedt,et al.  On the enzymic defects in hereditary tyrosinemia. , 1977, Proceedings of the National Academy of Sciences of the United States of America.

[216]  A. Goldberg,et al.  Intracellular protein degradation in mammalian and bacterial cells. , 1974, Annual review of biochemistry.

[217]  K. Nakao,et al.  Activity of Amino-laevulinic Acid Synthetase in Normal and Porphyric Human Livers , 1966, Nature.

[218]  S. Granick,et al.  EFFECT OF LEAD AND GENETIC FACTORS ON HEME BIOSYNTHESIS IN THE HUMAN RED CELL * , 1975, Annals of the New York Academy of Sciences.

[219]  M. Brodie,et al.  Haem biosynthesis in peripheral blood in erythropoietic protoporphyria , 1977, Clinical and experimental dermatology.

[220]  S. Matlin,et al.  The effect of the porphyrogenic compound, hexachlorobenzene, on the activity of hepatic uroporphyrinogen decarboxylase in the rat. , 1976, Clinical science and molecular medicine.

[221]  J. S. Woods.  Studies on the role of heme in the regulation of delta-aminolevulinic acid synthetase during fetal hepatic development. , 1974, Molecular pharmacology.

[222]  L. Flohé,et al.  Kinetics of metal chelatase from rat liver mitochondria , 1971, FEBS Letters.

[223]  S. Váradi.  Haematological Aspects in a Case of Erythropoietic Porphyria , 1958, British journal of haematology.

[224]  F. Lecocq,et al.  Porphyria precipitated by fasting. , 1967, The New England journal of medicine.

[225]  S. Granick,et al.  Studies in lead poisoning. I. Microanalysis of erythrocyte protoporphyrin levels by spectrophotometry in the detection of chronic lead intoxication in the subclinical range. , 1973, Biochemical medicine.

[226]  E. Stephens,et al.  Congenital Porphyria in Pigs , 1944, Nature.

[227]  Max Borst,et al.  Untersuchungen über Porphyrie : mit besonderer Berücksichtigung der Porphyria congenita , 1929 .

[228]  D. Nicholson,et al.  Erythrocyte protoporphyrin and iron uptake in erythropoietic protoporphyria. , 1971, Clinical science.

[229]  M. Moore,et al.  Effect of delta-aminolevulinic acid on frog nerve-muscle function. , 1978, Life sciences.

[230]  B. Haeger‐Aronsen.  ERYTHROPOIETIC PROTOPORPHYRIA. A NEW TYPE OF INBORN ERROR OF METABOLISM. , 1963, The American journal of medicine.

[231]  A. Theologides,et al.  A STUDY OF THE URINARY PORPHYRINS AND PORPHYRIN PRECURSORS IN PATIENTS WITH MALIGNANT DISEASE RECEIVING DIETHYLSTILBESTROL. , 1964, Metabolism: clinical and experimental.

[232]  R. Lemberg.  Porphyrins in Nature , 1954 .

[233]  G. Tobias.  CONGENITAL PORPHYRIA IN A CAT. , 1964, Journal of the American Veterinary Medical Association.

[234]  G. Elder,et al.  The porphyrias: a review. , 1972, Journal of clinical pathology.

[235]  S. Granick,et al.  The Enzymatic Synthesis of Porphyrins from Porphobilinogen. , 1953, Proceedings of the National Academy of Sciences of the United States of America.

[236]  G. Nigogosyan,et al.  Acquired toxic porphyria cutanea tarda due to hexachlorobenzene. Report of 348 cases caused by this fungicide. , 1963, JAMA.

[237]  L. Cantoni,et al.  Alteration of the porphyrin nucleus of cytochrome P-450 caused in the liver by treatment with allyl-containing drugs. Is the modified porphyrin N-substituted? , 1979, The Biochemical journal.

[238]  R. Zetterström,et al.  Tyrosinemia: An inborn error of tyrosine metabolism with cirrhosis of the liver and multiple renal tubular defects (de Toni-Debré-Fanconi syndrome) , 1965 .

[239]  A. Neuberger,et al.  Effect of splenectomy in a case of congenital porphyria. , 1952, Lancet.

[240]  J. Taljaard,et al.  Porphyrin Metabolism in Experimental Hepatic Siderosis in the Rat , 1972, British journal of haematology.

[241]  S. Granick,et al.  Stimulation of hemoglobin synthesis in chick blastoderms by certain 5beta androstane and 5beta pregnane steroids. , 1967, Proceedings of the National Academy of Sciences of the United States of America.

[242]  V. McKusick,et al.  Erythropoietic protoporphyria. A clinical and genetic study. , 1970, JAMA.

[243]  A. Meyer,et al.  The Anaemia of Erythropoietic Porphyria with the First Description of the Disease in an Elderly Patient , 1965 .

[244]  L. Taddeini,et al.  The clinical porphyrias. , 1968, Seminars in hematology.

[245]  F. Plum,et al.  Brain dysfunction in metabolic disorders , 1974 .

[246]  E. C. Amoroso,et al.  Congenital Porphyria in Bovines : First Living Cases in Britain , 1957, Nature.

[247]  F. T. Becker.  PORPHYRIA CUTANEA TARDA INDUCED BY ESTROGENS. , 1965, Archives of dermatology.

[248]  Z. Petryka,et al.  Effect of hematin in acute porphyric relapse. , 1973, Annals of internal medicine.

[249]  L. Bogorad.  The enzymatic synthesis of porphyrins from porphobilinogen. I. Uroporphyrin I. , 1958, The Journal of biological chemistry.

[250]  D. Bissell,et al.  Cytochrome P-450 heme and the regulation of hepatic heme oxygenase activity. , 1976, Archives of biochemistry and biophysics.

[251]  R. Jetton,et al.  THE ASSOCIATION OF LUPUS ERYTHEMATOSUS AND PORPHYRIA , 1970, The British journal of dermatology.

[252]  J. Strik.  Porphyrinogenic Action of Polyhalogenated Aromatic Compounds, With Special Reference to Porphyria and Environmental Impact , 1978 .

[253]  K. Anderson,et al.  Studies in porphyria. VIII. Relationship of the 5 alpha-reductive metabolism of steroid hormones to clinical expression of the genetic defect in acute intermittent porphyria. , 1979, The American journal of medicine.

[254]  M. Ua.  Intermittent acute porphyria. Clinical and biochemical studies of disordered heme biosynthesis. , 1973 .

[255]  G. Elder.  Identification of a group of tetracarboxylate porphyrins, containing one acetate and three propionate -substituents, in faeces from patients with symptomatic cutaneous hepatic porphyria and from rats with porphyria due to hexachlorobenzene. , 1972, The Biochemical journal.

[256]  C. J. Watson.  The problem of porphyria--some facts and questions. , 1960, The New England journal of medicine.

[257]  M. Doss,et al.  Uroporphyrinogen-Decarboxylase in Erythrocyten. Untersuchungen zum primären genetischen Enzymdefekt bei chronischer hepatischer Porphyrie , 1978 .

[258]  S. Lindstedt,et al.  ENZYMATIC STUDIES IN A CASE OF HEREDITARY TYROSINEMIA WITH HEPATOMA , 1969, Acta paediatrica Scandinavica.

[259]  T. Bird,et al.  Inherited deficiency of delta-aminolevulinic acid dehydratase. , 1979, American journal of human genetics.

[260]  S. Granick,et al.  Steroid induction of porphyrin synthesis in liver cell culture. I. Structural basis and possible physiological role in the control of heme formation. , 1967, The Journal of biological chemistry.

[261]  R. Schimke,et al.  The Genetic and Developmental Regulation of Hepatic δ-Aminolevulinate Dehydratase in Mice , 1969 .

[262]  R. Hess,et al.  Succinylacetone, a potent inhibitor of heme biosynthesis: effect on cell growth, heme content and delta-aminolevulinic acid dehydratase activity of malignant murine erythroleukemia cells. , 1979, Biochemical and biophysical research communications.

[263]  M. Seip,et al.  CONGENITAL ERYTHROPOIETIC PORPHYRIA WITH A HITHERTO UNDESCRIBED PORPHYRIN PATTERN , 1973 .

[264]  F. Margolis.  Regulation of porphyrin biosynthesis in the harderian gland of inbred mouse strains. , 1971, Archives of biochemistry and biophysics.

[265]  G. Collins,et al.  FACTORS AFFECTING THE EXCRETION OF PORPHYRIN PRECURSORS BY PATIENTS WITH ACUTE INTERMITTENT PORPHYRIA. I. THE EFFECT OF DIET. , 1964, Metabolism: clinical and experimental.

[266]  A. Kappas,et al.  LEAD AND METHYL MERCURY: EFFECTS OF ACUTE EXPOSURE ON CYTOCHROME P-450 AND THE MIXED FUNCTION OXIDASE SYSTEM IN THE LIVER , 1972, The Journal of experimental medicine.

[267]  P. Goldswain,et al.  The pattern of porphyrin isomer accumulation and excretion in symptomatic porphyria. , 1970, Clinical science.

[268]  J. S. Woods,et al.  δ-Aminolevulic Acid Synthetase from Fetal Rat Liver: Studies on the Partially Purified Enzyme , 1975 .

[269]  T. Chu,et al.  Porphyrin patterns in different types of porphyria. , 1967, Clinical chemistry.

[270]  A. Kappas,et al.  Studies in porphyria. I. A defect in the reductive transformation of natural steroid hormones in the hereditary liver disease, acute intermittent porphyria. , 1972 .

[271]  S. Granick,et al.  Mitochondrial coproporphyrinogen oxidase and protoporphyrin formation. , 1961, The Journal of biological chemistry.

[272]  B. Vernon‐roberts,et al.  Iron metabolism in porphyria cutanea tarda and in erythropoietic protoporphyria. , 1973, The Quarterly journal of medicine.

[273]  D. Weir,et al.  Suppression of Erythropoiesis by Alcohol , 1970, British medical journal.

[274]  T. Tephly,et al.  The role of substrates for glycine acyltransferase, and the role of sulfanilamide and acetate in the reversal of chemically-induced porphyria in the rat. , 1973, Enzyme.

[275]  C. Rimington,et al.  The excretion of porphyrin-peptide conjugates in porphyria variegata. , 1968, Clinical science.

[276]  J. Barboriak,et al.  Interstitial pneumonitis due to hypersensitivity to an organism contaminating a heating system. , 1971, Annals of internal medicine.

[277]  O. Jones,et al.  The structural organization of haem synthesis in rat liver mitochondria. , 1969, The Biochemical journal.

[278]  G. Laurent,et al.  Recherche de la pseudo-porphyrie cutanée tardive chez 100 hémodialysés , 1977 .

[279]  B. Kaplan.  delta-aminolevulinic acid synthetase from the particulate fraction of liver of porphyric rats. , 1971, Biochimica et biophysica acta.

[280]  P. Anderson,et al.  Purification and properties of delta-aminolevulinate dehydrase from human erythrocytes. , 1979, The Journal of biological chemistry.

[281]  R. Cantrill,et al.  δ-Aminolaevulinic acid is a potent agonist for GABA autoreceptors , 1979, Nature.

[282]  H. D. Barnes,et al.  Porphyria in Sweden and South Africa. , 1959, South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde.

[283]  H. Marver,et al.  Heme biosynthesis in intermittent acute prophyria: decreased hepatic conversion of porphobilinogen to porphyrins and increased delta aminolevulinic acid synthetase activity. , 1970, Proceedings of the National Academy of Sciences of the United States of America.

[284]  M. A. Everett,et al.  Diminished erythroid ferrochelatase activity in protoporphyria. , 1975, The Journal of laboratory and clinical medicine.

[285]  H. Fischer,et al.  Zur Kenntnis der natürlichen Porphyrine. II. Über das Turacin. , 1923 .

[286]  G. Getz,et al.  Intramitochondrial localization of δ-aminolaevulate synthetase and ferrochelatase in rat liver , 1969 .

[287]  A. Neuberger,et al.  Studies in congenital porphyria; the incorporation of 15N into the haem and glycine of haemoglobin. , 1950, The Biochemical journal.

[288]  F. Herbert.  Porphyrins excreted in various types of porphyria (with special reference to "pseudouroporphyrin"). , 1966, Clinica chimica acta; international journal of clinical chemistry.

[289]  I. Tsukamoto,et al.  The role of zinc with special reference to the essential thiol groups in delta-aminolevulinic acid dehydratase of bovine liver. , 1979, Biochimica et biophysica acta.

[290]  S. Balcerzak,et al.  Measurement of iron stores using deferoxamine. , 1968, Annals of internal medicine.

[291]  Y. Aoki.  Crystallization and characterization of a new protease in mitochondria of bone marrow cells. , 1978, The Journal of biological chemistry.

[292]  Z. Petryka,et al.  Uroporphyrinogen I synthase from human erythrocytes: separation, purification, and properties of isoenzymes. , 1979, Proceedings of the National Academy of Sciences of the United States of America.

[293]  Hiroshi Takahashi,et al.  A NEW FORM OF CONGENITAL HEMATOPORPHYRIA: OLIGOCHROMEMIA PORPHYRINURIA (MEGALOSPLENICA CONGENITA) , 1926 .

[294]  J. Waldenström.  The porphyrias as inborn errors of metabolism , 1957 .

[295]  D. Brenner,et al.  Study of factors causing excess protoporphyrin accumulation in cultured skin fibroblasts from patients with protoporphyria. , 1977, The Journal of clinical investigation.

[296]  A. Johnson,et al.  ENZYMIC FORMATION OF HAEMS AND OTHER METALLOPORPHYRINS. , 1964, Biochimica et biophysica acta.

[297]  C. Rimington,et al.  Diseases of porphyrin metabolism , 1962 .

[298]  C. J. Watson,et al.  Repression of the overproduction of porphyrin precursors in acute intermittent porphyria by intravenous infusions of hematin. , 1971, Proceedings of the National Academy of Sciences of the United States of America.

[299]  H. Marver,et al.  Decreased red cell uroporphyrinogen I synthetase activity in intermittent acute porphyria. , 1972, The Journal of clinical investigation.

[300]  J. Epstein,et al.  Porphyria cutanea tarda. Association with abnormal iron metabolism. , 1965, Archives of dermatology.

[301]  R. J. Long,et al.  In vitro inhibition of drug metabolism by metabolites of progesterone. , 1972, The Journal of pharmacology and experimental therapeutics.

[302]  W. Meinhof,et al.  Porphyrins in liver and urine in acute intermittent and chronic hepatic porphyrias. , 1971, South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde.

[303]  H. Roenigk,et al.  Estrogen-induced porphyria cutanea tarda. Report of three cases. , 1970, Archives of dermatology.

[304]  C. Peterson,et al.  Increased erythrocyte uroporphyrinogen-l-synthetase, delta-aminolevulinic acid dehydratase and protoporphyrin in hemolytic anemias. , 1977, The American journal of medicine.

[305]  J. Adamson,et al.  Steroids and hematopoiesis I. The effect of steroids on in vitro erythroid colony growth: Structure/activity relationships , 1976, Journal of cellular physiology.

[306]  B. Grandchamp,et al.  COPROPORPHYRINOGEN-OXIDASE DEFICIENCY IN HEREDITARY COPROPORPHYRIA , 1977, The Lancet.

[307]  M. Rabinowitz,et al.  Turnover of Cytochromes Labeled with δ-Aminolevulinic Acid-3H in Rat Liver , 1969 .

[308]  W. Levin,et al.  Incorporation of radioactive- -aminolevulinic acid into microsomal cytochrome P 450 : selective breakdown of the hemoprotein by allylisopropylacetamide and carbon tetrachloride. , 1972, Archives of biochemistry and biophysics.

[309]  W. Ostertag,et al.  Regulation of Heme Biosynthesis in Mouse Friend Virus-Transformed Cells in Culture , 1978 .

[310]  A. Neuberger,et al.  γ,δ-Dioxovalerate aminotransferase activity in Rhodopseudomonas spheroides , 1963 .

[311]  P. Mustard,et al.  Delta-aminolaevulinic acid synthetase activity in normal and porphyric human livers. , 1967, South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde.

[312]  C. Rimington,et al.  Erythropoietic protoporphyria. A new porphyria syndrome with solar urticaria due to protoporphyrinaemia. , 1961, Lancet.

[313]  G. Thompson,et al.  Changes in Harderian gland activity in the female golden hamster during the oestrous cycle, pregnancy and lactation. , 1979, The Biochemical journal.

[314]  B. Yoda,et al.  Mechanism of allylisopropylacetamide-induced increase of delta-aminolevulinate synthetase in liver mitochondria. IV. Accumulation of the enzyme in the soluble fraction of rat liver. , 1969, Archives of biochemistry and biophysics.

[315]  R. García,et al.  Porphyrin biosynthesis. VII. Porphyrinogen carboxy-lyase from avian erythrocytes. Purification and properties. , 1970, Biochimica et biophysica acta.

[316]  J. Lieberman,et al.  Presynaptic neuromuscular inhibition by porphobilinogen and porphobilin. , 1971, Proceedings of the National Academy of Sciences of the United States of America.

[317]  S. Granick,et al.  The Metabolism of Heme and Chlorophyll11Preparation of this manuscript was aided in part by funds from grant RG-4922 from the National Institutes of Health. , 1961 .

[318]  H. Marver,et al.  The molecular basis of the action of chloroquine in porphyria cutanea tarda. , 1973, The Journal of investigative dermatology.

[319]  C. Gray.  Isotope studies in porphyria. , 1952, British medical bulletin.

[320]  A. Turnbull.  IRON METABOLISM IN THE PORPHYRIAS , 1971, The British journal of dermatology.

[321]  C. Rimington,et al.  ISOTOPE STUDIES ON A CASE OF ERYTHROPOIETIC PROTOPORPHYRIA. , 1964, Clinical science.

[322]  S. Granick,et al.  HEME CONTROL ON THE SYNTHESIS OF DELTA‐AMINOLEVULINIC ACID SYNTHETASE IN CULTURED CHICK EMBRYO LIVER CELLS * , 1975, Annals of the New York Academy of Sciences.

[323]  S. Kramer.  IRON METABOLISM IN THE PORPHYRIAS. , 1963, South African journal of laboratory and clinical medicine. Suid-Afrikaanse tydskrif vir laboratorium- en kliniekwerk.

[324]  E. Dowdle,et al.  THE DIAGNOSTIC IMPORTANCE OF FAECAL PORPHYRINS IN THE DIFFERENTIATION OF THE PORPHYRIAS. II. VALUES IN THE CUTANEOUS PORPHYRIAS. , 1963, South African journal of laboratory and clinical medicine. Suid-Afrikaanse tydskrif vir laboratorium- en kliniekwerk.

[325]  S. D. Wainwright,et al.  REGULATION OF THE INITIATION OF HEMOGLOBIN SYNTHESIS IN THE BLOOD ISLAND CELLS OF CHICK EMBRYOS. II. EARLY ONSET AND STIMULATION OF HEMOGLOBIN FORMATION INDUCED BY EXOGENOUS δ-AMINOLEVULINIC ACID , 1967 .

[326]  S. Granick,et al.  Pbrphyrin biosynthesis in erythrocytes. II. Enzymes converting gamma-aminolevulinic acid to coproporphyrinogen. , 1958, The Journal of biological chemistry.

[327]  S. Beale,et al.  The Biosynthesis of δ-Aminolevulinic Acid in Higher Plants: II. Formation of 14C-δ-Aminolevulinic Acid from Labeled Precursors in Greening Plant Tissues 1 , 1974 .

[328]  R. Summerly,et al.  Cutaneous Hepatic Porphyria and Oestrogens , 1966, British medical journal.

[329]  D. Bickers,et al.  A microassay for uroporphyrinogen I synthase, one of three abnormal enzyme activities in acute intermittent porphyria, and its application to the study of the genetics of this disease. , 1974, Proceedings of the National Academy of Sciences of the United States of America.

[330]  J. Lawrence,et al.  Phototoxic blisters from high frusemide dosage , 1976, The British journal of dermatology.

[331]  R. Levere.  Stilbestrol-Induced Porphyria: Increase in Hepatic δ-Aminolevulinic Acid Synthetase , 1966 .

[332]  K. Richards,et al.  The Quaternary Structure of δ-Aminolevulinic Acid Dehydratase from Bovine Liver , 1974 .

[333]  Glenn Bl,et al.  Congenital porphyria in the domestic cat (Felis catus): preliminary investigations on inheritance pattern. , 1968 .

[334]  G. C. Mills,et al.  Coexistent Systemic Lupus Erythematosus and Porphyria , 1966 .

[335]  Z. Petryka,et al.  Location of iron and porphyrin in the liver in porphyria cutanea tarda. , 1978, Acta histochemica.

[336]  H. Marver,et al.  Delta-aminolevulinic acid synthetase. II. Induction in rat liver. , 1966, The Journal of biological chemistry.

[337]  S. D. Wainwright,et al.  REGULATION OF THE INITIATION OF HEMOGLOBIN SYNTHESIS IN THE BLOOD ISLAND CELLS OF CHICK EMBRYOS: I. QUALITATIVE STUDIES OF THE EFFECTS OF ACTINOMYCIN AND δ-AMINOLEVULINIC ACID , 1966 .

[338]  L. Heilmeyer,et al.  Die Störung der Porphyrinsynthese bei der Güntherschen Porphyria congenita , 1965 .

[339]  S. Joubert,et al.  Porphyria in the African: a study of 100 cases. , 1961, The Quarterly journal of medicine.

[340]  M. Losowsky,et al.  Haem synthetase activity of human blood cells. , 1970, Clinica chimica acta; international journal of clinical chemistry.

[341]  E. C. Weinbach,et al.  Porphyrin synthesis and mitochondrial respiration in acute intermittent porphyria: studies using cultured human fibroblasts. , 1975, Journal of Laboratory and Clinical Medicine.

[342]  R. P. Thompson,et al.  Cutaneous porphyria due to a malignant primary hepatoma. , 1970, Gastroenterology.

[343]  P. Cornford.  Transformation of porphobilinogen into porphyrins by preparations from human erythrocytes. , 1964, The Biochemical journal.

[344]  F. Matteis.  Loss of microsomal components in drug-induced liver damage, in cholestasis and after administration of chemicals which stimulate heme catabolism , 1978 .

[345]  J. Epstein,et al.  Lupus erythematosus and porphyria. Coexistence in seven patients. , 1973, Archives of dermatology.

[346]  T. Zimmerman,et al.  Onset of manifestations of hepatic porphyria in relation to the influence of female sex hormones. , 1966, Archives of internal medicine.

[347]  J. Goldstein,et al.  Hepatic porphyria induced by 2,3,7,8-tetrachlorodibenzo-p-dioxin in the mouse. , 1973, Research communications in chemical pathology and pharmacology.

[348]  S. Granick,et al.  THE OCCURRENCE AND DETERMINATION OF δ-AMINOLEVULINIC ACID AND PORPHOBILINOGEN IN URINE , 1956 .

[349]  S. Sassa,et al.  Hemin control of heme biosynthesis in mouse Friend virus-transformed erythroleukemia cells in culture. , 1978, The Journal of biological chemistry.

[350]  Kevin M. Smith,et al.  Porphyrins and metalloporphyrins. , 1975 .

[351]  R. Winchester,et al.  Stimulators and inhibitors of hepatic porphyrin formation in human sera. , 1974, The Journal of clinical investigation.

[352]  R. Labbé,et al.  Effects of diethyl-1, 4-dihydro-2, 4,6-trimethylpyridine-3,5-dicarboxylate on the metabolism of porphyrins and iron. , 1963, The Journal of biological chemistry.

[353]  F. Cole,et al.  Iron deficiency prevents liver toxicity of 2,3,7,8-tetrachlorodibenzo-p-dioxin. , 1979, Science.

[354]  L. H. Grimme,et al.  Tetrapyrrole biosynthesis in algae and higher plants: a discussion of the importance of the 5-aminolaevulinate synthase and the dioxovalerate transaminase pathways in the biosynthesis of chlorophyll. , 1978, The International journal of biochemistry.

[355]  T. Tephly,et al.  Effect of drugs on heme synthesis in the liver. , 1971, Metabolism: clinical and experimental.

[356]  A. Batlle,et al.  Porphyrin biosynthesis. 8. Avian erythrocyte porphobilinogen deaminase-uroporphyrinogen 3 cosynthetase, its purification, properties and the separation of its components. , 1971, Biochimica et biophysica acta.

[357]  S. Granick,et al.  THE INDUCTION OF delta-AMINOLEVULINIC ACID SYNTHETASE in vivo IN CHICK EMBRYO LIVER BY NATURAL STEROIDS. , 1968, Proceedings of the National Academy of Sciences of the United States of America.

[358]  S. Schwartz,et al.  A Simple Test for Urinary Porphobilinogen.∗ , 1941 .

[359]  H. Marver,et al.  Soluble -aminolevulinic acid synthetase of rat liver. II. Studies related to the mechanism of enzyme action and hemin inhibition. , 1972, The Journal of biological chemistry.

[360]  S. Zail,et al.  Porphyrin Metabolism in Experimental Hepatic Siderosis in the Rat , 1970, British journal of haematology.

[361]  S. Granick,et al.  The occurrence of substances in human plasma capable of inducing the enzyme delta-aminolevulinate synthetase in liver cells. , 1969, Proceedings of the National Academy of Sciences of the United States of America.

[362]  F. De Matteis,et al.  A difference between two strains of rats in their liver non-haem iron content and in their response to the porphyrogenic effect of hexachlorobenzene. , 1979, Chemico-biological interactions.

[363]  D. Tschudy,et al.  Effect of 3-amino-1, 2, 4-triazole on delta-amino-levulinic acid dehydrase activity. , 1957, Science.

[364]  E. Y. Levin.  Uroporphyrinogen m Cosynthetase in Bovine Erythropoietic Porphyria , 1968, Science.

[365]  D. Bickers,et al.  Studies on the inheritance of human erythrocyte delta-aminolevulinate dehydratase and uroporphyrinogen synthetase. , 1973, Enzyme.

[366]  F. De Matteis,et al.  Drug-induced conversion of liver haem into modified porphyrins. Evidence for two classes of products. , 1980, The Biochemical journal.

[367]  H. Fischer,et al.  Über Uroporphyrin aus Muschelschalen. , 1932 .

[368]  G. Elder.  Differentiation of porphyria cutanea tarda symptomatica from other types of porphyria by measurement of isocoproporphyrin in faeces. , 1975, Journal of clinical pathology.

[369]  S. Joubert,et al.  PORPHYRIA IN THE AFRICAN1 , 1961 .

[370]  V. Herbert,et al.  SUPPRESSION HEMATOPOIESIS BY ETHANOL. , 1964, The Journal of clinical investigation.

[371]  R. J. Porra,et al.  Studies on ferrochelatase. 1. Assay and properties of ferrochelatase from a pig-liver mitochondrial extract. , 1963, The Biochemical journal.

[372]  S. Piomelli,et al.  Erythropoietic protoporphyria and lead intoxication: the molecular basis for difference in cutaneous photosensitivity. I. Different rates of disappearance of protoporphyrin from the erythrocytes, both in vivo and in vitro. , 1975, The Journal of clinical investigation.

[373]  S. Sassa,et al.  Steroid induction of delta-aminolevulinic acid synthase and porphyrins in liver. Structure-activity studies and the permissive effects of hormones on the induction process. , 1979, The Journal of biological chemistry.

[374]  G. Padmanaban,et al.  Studies on the induction of -aminolevulinic acid synthetase iun mouse liver. , 1973, Archives of biochemistry and biophysics.

[375]  C. Rimington,et al.  II. Biochemical Investigations , 1972 .

[376]  C. Magnussen,et al.  A red cell enzyme method for the diagnosis of acute intermittent porphyria. , 1974, Blood.

[377]  S. Granick,et al.  Steroid induction of porphyrin synthesis in liver cell culture. , 1967, Transactions of the Association of American Physicians.

[378]  Rosemberg Ja,et al.  Effect of diet on induction of experimental porphyria. , 1961 .

[379]  G. Elder.  Porphyrin metabolism in porphyria cutanea tarda. , 1977, Seminars in hematology.

[380]  S. Granick,et al.  Porphyrin biosynthesis in erythrocytes. III. Uroporphyrinogen and its decarboxylase. , 1958, The Journal of biological chemistry.

[381]  W. H. Elliott,et al.  Purification and properties of solubilized mitochondrial -aminolevulinic acid synthetase and comparison with the cytosol enzyme. , 1972, The Journal of biological chemistry.

[382]  W. Epstein,et al.  Porphyria-like cutaneous changes induced by tetracycline hydrochloride photosensitization. , 1976, Archives of dermatology.

[383]  R. Schmid,et al.  Erythropoietic (congenital) porphyria: A rare abnormality of the normoblasts. , 1955, Blood.

[384]  E. Lipton,et al.  Effect of splenectomy on porphyria erythropoietica. , 1955, Pediatrics.

[385]  H. Sawada,et al.  Effect of lipid on protoheme ferro-lyase. , 1969, Biochimica et biophysica acta.

[386]  M. Brodie,et al.  The treatment of acute intermittent porphyria with laevulose. , 1977, Clinical science and molecular medicine.

[387]  B. Shanley,et al.  Prophyrin precursors in blood, urine and cerebrospinal fluid in acute porphyria. , 1977, South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde.

[388]  C. Rimington,et al.  A study of the porphyrins excreted in the urine by a case of congenital porphyria. , 1951, The Biochemical journal.

[389]  H. Heath,et al.  Intermediates in the Biosynthesis of Porphyrins from Porphobilinogen by Rhodopseudomonas spheroides , 1958, Nature.

[390]  A. Kappas,et al.  Studies in porphyria. II. Evidence for a deficiency of steroid delta-4-5-alpha-reductase activity in acute intermittent porphyria. , 1973 .

[391]  J. Bleiberg,et al.  Industrially Acquired Porphyria , 1964 .

[392]  M. Higuchi,et al.  THE PURIFICATION AND PROPERTIES OF UROPORPHYRINOGEN I SYNTHASES AND UROPORPHYRINOGEN III COSYNTHASE. INTERACTIONS BETWEEN THE ENZYMES * , 1975, Annals of the New York Academy of Sciences.

[393]  P. Lundin,et al.  Iron storage in porphyria cutanea tarda. , 2009, Acta medica Scandinavica.

[394]  F. Takaku,et al.  -Aminolevulinic acid synthetase activity of human bone marrow erythroid cells in various hematological disorders. , 1972, The Tohoku journal of experimental medicine.

[395]  L. Taddeini,et al.  PRESENT STATUS OF THE EHRLICH ALDEHYDE REACTION FOR URINARY PORPHOBILINOGEN. , 1964, JAMA.

[396]  R. Cox,et al.  THE PRIMARY ENZYME DEFECT IN HEREDITARY COPROPORPHYRIA , 1976, The Lancet.

[397]  H. Krook,et al.  Follow-up studies on an unselected ten-year material of 360 patients with liver cirrhosis in one community. , 2009, Acta medica Scandinavica.

[398]  H. H. Hoyt,et al.  Some studies of the comparative biology of human and bovine porphyria erythropoietica. , 1958, Transactions of the Association of American Physicians.

[399]  R. Winkelmann.  The Metabolic Basis of Inherited Diseases , 1972 .

[400]  T. Chu,et al.  Porphyrins from congenitally porphyric (pink-tooth) cattle. , 1962, The Biochemical journal.

[401]  A. Smith,et al.  Decarboxylation of porphyrinogens by rat liver uroporphyrinogen decarboxylase. , 1979, The Biochemical journal.

[402]  D. Tschudy.  BIOCHEMICAL LESIONS IN PORPHYRIA. , 1965, JAMA.

[403]  I. London,et al.  CONTROL OF HEME SYNTHESIS BY FEEDBACK INHIBITION. , 1965, Biochemical and biophysical research communications.

[404]  G. Romeo,et al.  Uroporphyrinogen decarboxylase from mouse spleen. , 1971, Biochimica et biophysica acta.

[405]  E. Kang,et al.  Hereditary tyrosinemia and abnormal pyrrole metabolism. , 1970, The Journal of pediatrics.

[406]  A. Barbuto,et al.  An inherited enzymatic defect in porphyria cutanea tarda: decreased uroporphyrinogen decarboxylase activity. , 1976, The Journal of clinical investigation.

[407]  H. Marver,et al.  ACUTE INTERMITTENT PORPHYRIA: THE FIRST "OVERPRODUCTION DISEASE" LOCALIZED TO A SPECIFIC ENZYME. , 1965, Proceedings of the National Academy of Sciences of the United States of America.

[408]  J. Ross,et al.  Induction of globin mRNA accumulation by hemin in cultured erythroleukemic cells , 1976, Cell.

[409]  J. Kaneko.  ERYTHROKINETICS AND IRON METABOLISM IN BOVINE PORPHYRIA ERYTHROPOIETICA * , 1963, Annals of the New York Academy of Sciences.

[410]  L. Heilmeyer,et al.  [ERYTHROPOIETIC PROTOPORPHYRIA. PROTOPORPHYRINEMIC PHOTODERMATOSIS]. , 1964, Deutsche medizinische Wochenschrift.

[411]  C. Finch,et al.  Iron metabolism; iron stores in man as measured by phlebotomy. , 1952, The Journal of clinical investigation.

[412]  N. Kaplowitz,et al.  Isolation of erythrocytes with normal protoporphyrin levels in erythropoietic protoporphyria. , 1968, The New England journal of medicine.

[413]  E. Cayanis,et al.  Reduced ferrochelatase activity in fibroblasts from patients with porphyria variegata , 1979, American journal of hematology.

[414]  J. Pearn.  A Clinical and Genetic Study , 1978 .

[415]  B. Grandchamp,et al.  The spectrophotometric determination of uroporphyrinogen I synthetase activity. , 1976, Clinica chimica acta; international journal of clinical chemistry.

[416]  P. Mustajoki.  Red cell uroporphyrinogen I synthetase in acute intermittent porphyria. , 1976, Annals of clinical research.

[417]  Thomas B. Fitzpatrick,et al.  Dermatology in general medicine , 1971 .

[418]  H. Marver,et al.  Erythropoietic protoporphyria: evidence for multiple sites of excess protoporphyrin formation. , 1971, The Journal of clinical investigation.

[419]  T. Yoshinaga,et al.  Coproporphyrinogen oxidase. I. Purification, properties, and activation by phospholipids. , 1980, The Journal of biological chemistry.

[420]  W. Polglase,et al.  Site of glucose repression of heme biosynthesis , 1974, FEBS letters.

[421]  A. Battle,et al.  Porphyrin biosynthesis. VI. Separation and purification of porphobilinogen deaminase and uroporphyrinogen isomerase from cow liver. Porphobilinogenase an allosteric enzyme. , 1969, Biochimica et biophysica acta.

[422]  B. Grandchamp,et al.  DETECTION OF INTERMITTENT ACUTE PORPHYRIA TRAIT IN CHILDREN , 1976, The Lancet.

[423]  Elder Gh.  The metabolism of porphyrins of the isocoproporphyrin series. , 1974 .

[424]  D. Bickers,et al.  Porphyria cutanea tarda. Clinical features and laboratory findings in 40 patients. , 1979, The American journal of medicine.

[425]  S. K. Jørgensen.  Congenital porphyria in swine and cattle in Denmark. , 1955 .

[426]  G. Stathers,et al.  Hereditary Coproporphyria: Study of a Swedish Family , 1968 .

[427]  R. Labbé,et al.  Metal specificity of the ironprotoporphyrin chelating enzyme from rat liver. , 1961, Biochimica et biophysica acta.

[428]  H. Ippen.  Treatment of porphyria cutanea tarda by phlebotomy. , 1977, Seminars in hematology.

[429]  L. Eales.  PORPHYRIA AS SEEN IN CAPE TOWN. A SURVEY OF 250 PATIENTS AND SOME RECENT STUDIES. , 1963, South African journal of laboratory and clinical medicine. Suid-Afrikaanse tydskrif vir laboratorium- en kliniekwerk.

[430]  S. Granick,et al.  Effects by heme, insulin, and serum albumin on heme and protein synthesis in chick embryo liver cells cultured in a chemically defined medium, and a spectrofluorometric assay for porphyrin composition. , 1975, The Journal of biological chemistry.

[431]  M. Seip,et al.  Congenital erythropoietic porphyria:A family study , 1973, Clinical genetics.

[432]  R. Nicoll.  The interaction of porphyrin precursors with GABA receptors in the isolated frog spinal cord. , 1976, Life sciences.

[433]  A. Redeker,et al.  The "glucose effect" in erythropoietic protoporphyria. , 1968, Archives of internal medicine.

[434]  N. Hayashi,et al.  Purification and some properties of delta-aminolevulinate synthase from the rat liver cytosol fraction and immunochemical identity of the cytosolic enzyme and the mitochondrial enzyme. , 1980, The Journal of biological chemistry.

[435]  L. Eales,et al.  Enzymatic defects in hepatic porphyria. Preliminary observations in patients with porphyria cutanea tarda and variegate porphyria. , 1973, Enzyme.

[436]  E. Astrup.  Family studies on the activity of uroporphyrinogen I synthase in diagnosis of acute intermittent porphyria. , 1978, Clinical science and molecular medicine.

[437]  M. Doss,et al.  Uroporphyrinogen-Synthase in Erythrocyten bei akuter intermittierender Porphyrie: Neue pathobiochemische Aspekte , 1978 .

[438]  D. Bickers,et al.  Studies on porphyrin-heme biosynthesis in organotypic cultures of chick dorsal root ganglion. I. Observations on neuronal and non-neuronal elements. , 1978, Journal of neuropathology and experimental neurology.

[439]  R. Aldrich,et al.  Photosensitive or congenital porphyria with hemolytic anemia. I. Clinical and fundamental studies before and after splenectomy. , 1951, Blood.

[440]  H. Marver,et al.  Intermittent acute porphyria--demonstration of a genetic defect in porphobilinogen metabolism. , 1972, The New England journal of medicine.

[441]  M. Moore,et al.  17-Oxosteroid conjugates in plasma and urine of patients with acute intermittent porphyria. , 1974, Clinical science and molecular medicine.

[442]  P. Voytek,et al.  Liver and red-cell porphobilinogen synthase in the adult and fetal guinea pig. , 1974, Biochimica et biophysica acta.

[443]  L. C. San Martín de Viale,et al.  Porphyrins and porphyrinogen carboxy-lase in hexachlorobenzene-induced porphyria. , 1977, The Biochemical journal.

[444]  E. Y. Levin.  Enzymatic properties of uroporphyrinogen 3 cosynthetase. , 1971, Biochemistry.