COPPER DEFICIENCY IN THE DEVELOPING RAT BRAIN: A POSSIBLE MODEL FOR MENKES’ STEELY‐HAIR DISEASE

Abstract— In comparison to controls, copper‐deficient suckling rats showed an appreciable decrease in body growth, a slight decrease in whole brain and cerebellar growth, and a highly significant decrease in myelination based on the activity of cerebellar 2′,3′‐cyclic nucleotide 3′‐phosphohydrolase—a myelin enriched protein. Specific effects of a fivefold reduction in the copper content of brain were seen in a drastic decrease in cerebellar cytochrome c oxidase and smaller but significant drops in cerebellar super‐oxide dismutase and brain norepinephrine concn. These observations are discussed with respect to the neuropathology and biochemistry of Menkes’ steely‐hair disease, a sex‐linked recessive disorder in humans characterized by copper deficiency.

[1]  W. W. Wells,et al.  Improved rapidity and precision in the determination of brain 2',3'-cyclic nucleotide 3'-phosphohydrolase. , 1973, Analytical biochemistry.

[2]  H. F. Deutsch,et al.  The levels of superoxide dismutase and catalase in human tissues as determined immunochemically. , 1973, Clinica chimica acta; international journal of clinical chemistry.

[3]  S. D. Murphy,et al.  Effect of excess and deficient copper intake on rat liver microsomal enzyme activity. , 1973, Biochemical pharmacology.

[4]  S. Aust,et al.  The role of superoxide and singlet oxygen in lipid peroxidation promoted by xanthine oxidase. , 1973, Biochemical and biophysical research communications.

[5]  H. Lou,et al.  Brain-copper concentration in Menkes' disease. , 1973, Lancet.

[6]  H. Chase.  THE EFFECTS OF INTRAUTERINE AND POSTNATAL UNDERNUTRITION ON NORMAL BRAIN DEVELOPMENT * , 1973, Annals of the New York Academy of Sciences.

[7]  J. Howell,et al.  Lesions produced by copper deficiency in neonate and older rats , 1973, British Journal of Nutrition.

[8]  J. Fee,et al.  Evidence that superoxide dismutase plays a role in protecting red blood cells against peroxidative hemolysis. , 1972, Biochemical and biophysical research communications.

[9]  P E Campbell,et al.  MENKES'S KINKY HAIR SYNDROME , 1972, Pediatrics.

[10]  B. J. Stevens,et al.  Menkes' kinky-hair syndrome. , 1972, Lancet.

[11]  J. French,et al.  Trichopoliodystrophy. I. Report of a case and biochemical studies. , 1972, Archives of neurology.

[12]  Owen Ca.  Metabolism of copper 67 by the copper-deficient rat. , 1971, The American journal of physiology.

[13]  J. Spangler,et al.  Neonatal copper deficiency. , 1971, The New England journal of medicine.

[14]  V. Reeve,et al.  Copper deficiency in the rat. Effect on liver and brain lipids. , 1971, The Australian journal of experimental biology and medical science.

[15]  R. Wurtman,et al.  Perinatal Undernutrition: Accumulation of Catecholamines in Rat Brain , 1971, Science.

[16]  V. Reeve,et al.  Copper deficiency in the rat. Effect of synthesis of phospholipids. , 1971, The Australian journal of experimental biology and medical science.

[17]  Y. Takahashi,et al.  Spectrophotometric determination of copper with N,N,N',N'-tetraethylthiuram disulfide and an application of this method for studies on subcellular distribution of copper in rat brain. , 1970, Analytical biochemistry.

[18]  I. Fridovich,et al.  Superoxide dismutase. An enzymic function for erythrocuprein (hemocuprein). , 1969, The Journal of biological chemistry.

[19]  G. Drummond,et al.  Studies on 2',3'-cyclic nucleotide-3'-phosphohydrolase from brain. , 1969, Canadian journal of biochemistry.

[20]  J. Howell,et al.  The effect of copper deficiency on reproduction in the female rat , 1969, British Journal of Nutrition.

[21]  G. Everson,et al.  Chemical and morphological changes in the brains of copper-deficient guinea pigs. , 1968 .

[22]  K. Taylor,et al.  The fluorometric assay of catecholamines and related compounds: improvements and extensions to the hydroxyindole technique. , 1968, Analytical biochemistry.

[23]  T. Kurihara,et al.  THE REGIONAL AND SUBCELLULAR DISTRIBUTION OF 2′,3′‐CYCLIC NUCLEOTIDE 3′‐PHOSPHOHYDROLASE IN THE CENTRAL NERVOUS SYSTEM , 1967, Journal of neurochemistry.

[24]  G. Everson,et al.  Copper deficiency in the guinea pig. , 1967, The Journal of nutrition.

[25]  H. Agrawal,et al.  Changes in monoamines of rat brain during postnatal ontogeny. , 1966, Biochimica et biophysica acta.

[26]  J. O'brien,et al.  KINKY HAIR DISEASE: II. BIOCHEMICAL STUDIES , 1966 .

[27]  S. Kamoshita,et al.  KINKY HAIR DISEASE: I. CLINICAL AND PATHOLOGICAL FEATURES , 1966 .

[28]  I. H. Tipton,et al.  Essential trace metals in man: copper. , 1966, Journal of chronic diseases.

[29]  S. Kaufman,et al.  3,4-dihydroxyphenylethylamine beta-hydroxylase. Physical properties, copper content, and role of copper in the catalytic acttivity. , 1965, The Journal of biological chemistry.

[30]  T. Yonetani.  Studies on cytochrome c peroxidase. II. Stoichiometry between enzyme, H2O2, and ferrocytochrome c and enzymic determination of extinction coefficients of cytochrome c. , 1965, The Journal of biological chemistry.

[31]  R. M. Fleming,et al.  SINGLE EXTRACTION METHOD FOR THE SIMULTANEOUS FLUOROMETRIC DETERMINATION OF SEROTONIN, DOPAMINE, AND NOREPINEPHRINE IN BRAIN. , 1965, Analytical chemistry.

[32]  C. F. Mills,et al.  CYTOCHROME OXIDASE DEFICIENCY IN THE MOTOR NEURONES OF COPPER-DEFICIENT LAMBS: A HISTOCHEMICAL STUDY. , 1965, Research in veterinary science.

[33]  J. Dobbing.  The influence of early nutrition on the development and myelination of the brain , 1964, Proceedings of the Royal Society of London. Series B. Biological Sciences.

[34]  C. F. Mills,et al.  Copper concentration and cytochrome-oxidase and ribonuclease activities in the brains of copper-deficient lambs. , 1962, The Biochemical journal.

[35]  M. Alter,et al.  A sex-linked recessive disorder with retardation of growth, peculiar hair, and focal cerebral and cerebellar degeneration. , 1962, Pediatrics.

[36]  B. L. O’dell,et al.  Connective Tissue Defect in the Chick Resulting from Copper Deficiency.∗ , 1961, Proceedings of the Society for Experimental Biology and Medicine. Society for Experimental Biology and Medicine.

[37]  P. J. Warren,et al.  The distribution of copper in human brain. , 1960, Brain : a journal of neurology.

[38]  Sidney Udenfriend,et al.  LOCALIZATION OF DOPAMINE‐β‐OXIDASE IN BRAIN , 1959 .

[39]  N. F. Shimp,et al.  Copper in cytochrome c oxidase. , 1959, Nutrition reviews.

[40]  A. Davison,et al.  The copper content and cytochrome oxidase activity of tissues from normal and swayback lambs. , 1959, The Biochemical journal.

[41]  A. Hughes Bryan,et al.  Trace Elements in Human and Animal Nutrition , 1957 .

[42]  J. Folch,et al.  CEREBROCUPREIN I. A COPPER‐CONTAINING PROTEIN ISOLATED FROM BRAIN , 1957, Journal of neurochemistry.

[43]  K. Rees,et al.  The biochemistry of copper deficiency - II. Synthetic processes , 1956, Proceedings of the Royal Society of London. Series B - Biological Sciences.

[44]  K. Rees,et al.  The biochemistry of copper deficiency I. Enzymological disturbances, blood chemistry and excretion of amino-acids , 1956, Proceedings of the Royal Society of London. Series B - Biological Sciences.

[45]  E. Frick,et al.  [Development of the brain in copper-deficient rat]. , 1953, Klinische Wochenschrift.

[46]  E. B. Hart,et al.  IRON IN NUTRITION VII. COPPER AS A SUPPLEMENT TO IRON FOR HEMOGLOBIN BUILDING IN THE RAT , 1928 .

[47]  J. Trotter,et al.  Crystal Structure of 1-Cyanotetracyclo[3.3.1.13,7.03,7]decane , 1973 .

[48]  A. Field,et al.  Effects of Cu deficiency and of dietary Cu intake on the distribution of 64Cu in the rat. , 1973, Journal of comparative pathology.

[49]  V. Peden,et al.  Copper deficiency in long-term parenteral nutrition. , 1972, The Journal of pediatrics.

[50]  W. A. Kelly,et al.  Neural lesions in the offspring of female rats fed a copper-deficient diet. , 1969, The Journal of nutrition.

[51]  M. H. Aprison,et al.  Microdetermination of norepinephrine, 3,4-dihydroxyphenylethylamine, and 5-hydroxyyptamine from single extracts of specific rat brain areas. , 1969, Analytical biochemistry.