Biochemistry of the elastic fibers in normal connective tissues and its alterations in diseases.

The elastic fibers present in various connective tissues of the body are responsible for physiologic elasticity of the organs. These fibers consist of 2 distinct components, elastin and the elastic fiber microfibrils. Controlled synthesis and balanced interaction of these 2 components are essential for normal fibrillogenesis. The intracellular biosynthesis of elastin by connective tissue cells, such as smooth muscle cells, involves assembly of the polypeptide chains on the membrane-bound ribosomes, hydroxylation of some prolyl residues to hydroxyproline, and secretion of the polypeptides packaged in Golgi vacuoles. In the extracellular space the elastin molecules assemble into fiber structures which are stabilized by the synthesis of complex covalent cross-links, desmosines. Recently, aberrations in the structure or metabolism of elastin have been detected in a variety of heritable and acquired diseases affecting skin and other connective tissues. These conditions include pseudoxanthoma elasticum, cutis laxa, and elastosis perforans serpiginosa, as well as arteriosclerosis and other degenerative changes of the vascular connective tissues.

[1]  D. Urry,et al.  Molecular Perspectives of Vascular Wall Structure and Disease: The Elastic Component , 2015, Perspectives in biology and medicine.

[2]  R. Mecham,et al.  Proelastin. Synthesis in cultured smooth muscle cells. , 1978, The Journal of biological chemistry.

[3]  D. Jackson,et al.  The identification of glycoproteins associated with elastic-tissue microfibrils. , 1978, The Biochemical journal.

[4]  D. Prockop,et al.  Synthesis of an elastin component of molecular weight about 70,000 by polysomes from chick embryo aortas. , 1978, Archives of biochemistry and biophysics.

[5]  E. Johnson,et al.  Apatite crystals in pseudoxanthoma elasticum: a combined study using electron microscopy and selected area diffraction analysis. , 1977, The Journal of investigative dermatology.

[6]  R. Mecham,et al.  Trypsin-like neutral protease associated with soluble elastin. , 1977, Biochemistry.

[7]  D. Archer,et al.  A Study of Four Cases of Pseudoxanthoma Elasticum , 1977, Journal of cutaneous pathology.

[8]  B. Starcher.  Determination of the elastin content of tissues by measuring desmosine and isodesmosine. , 1977, Analytical biochemistry.

[9]  D. Prockop,et al.  Synthesis of elastin in aortas from chick embryos. Conversion of newly secreted elastin to cross-linked elastin without apparent proteolysis of the molecule. , 1977, Biochemistry.

[10]  M. Lefevre,et al.  Putative forms of soluble elastin and their relationship to the synthesis of fibrous elastin. , 1977, Biochemical and biophysical research communications.

[11]  R. Mecham,et al.  A high molecular weight species of soluble elastin. , 1976, Biochemical and biophysical research communications.

[12]  R. Siegel,et al.  Collagen cross-linking. Purification and substrate specificity of lysyl oxidase. , 1976, The Journal of biological chemistry.

[13]  J. Rosenbloom,et al.  Inhibition of proline hydroxylation does not inhibit secretion of tropoelastin by chick aorta cells , 1976, FEBS letters.

[14]  J. Rosenbloom,et al.  Biosynthesis and secretion of tropoelastin by chick aorta cells. , 1976, Biochemical and biophysical research communications.

[15]  R. Ross,et al.  A presumptive subunit of elastic fiber microfibrils secreted by arterial smooth-muscle cells in culture. , 1976, European journal of biochemistry.

[16]  J. Uitto,et al.  Synthesis of elastin and procallagen by cells from embryonic aorta. Differences in the role of hydroxyproline and the effects of proline analogs on the secretion of the two proteins. , 1976, Archives of biochemistry and biophysics.

[17]  R. Ross,et al.  The smooth muscle cell. III. Elastin synthesis in arterial smooth muscle cell culture , 1976, The Journal of cell biology.

[18]  R. Page,et al.  Demonstration of a precursor-product relationship between soluble and cross-linked elastin, and the biosynthesis of the desmosines in vitro. , 1976, The Journal of biological chemistry.

[19]  J. Travis,et al.  Human leukocyte granule elastase: rapid isolation and characterization. , 1976, Biochemistry.

[20]  J. Uitto,et al.  Defects in the biochemistry of collagen in diseases of connective tissue. , 1976, The Journal of investigative dermatology.

[21]  C. Franzblau,et al.  The synthesis of connective tissue protein in smooth muscle cells. , 1976, Biochimica et biophysica acta.

[22]  R. Shapiro,et al.  Isolation of soluble elastin from lathyritic chicks. Comparison to tropoelastin from copper deficient pigs. , 1975, Biochemistry.

[23]  M. Haniu,et al.  Chick tropoelastin. Isolation and partial chemical characterization. , 1975, Biochemical and biophysical research communications.

[24]  S Gordon,et al.  Elastase secretion by stimulated macrophages. Characterization and regulation , 1975, The Journal of experimental medicine.

[25]  K. Hashimoto,et al.  Cutis laxa. Ultrastructural and biochemical studies. , 1975 .

[26]  L. Sandberg,et al.  Tropoelastin purification: improvements using enzyme inhibitors. , 1975, Analytical biochemistry.

[27]  P. Fialkow,et al.  Pseudoxanthoma elasticum. An underdiagnosed genetically heterogeneous disorder with protean manifestations. , 1974, Archives of internal medicine.

[28]  L B Sandberg,et al.  Isolation and characterization of cross-linked peptides from elastin. , 1974, The Journal of biological chemistry.

[29]  W. Huffer,et al.  Pseudoxanthoma elasticum: dermal polyanions and the mineralization of elastic fibers. , 1974, Laboratory investigation; a journal of technical methods and pathology.

[30]  B. Sykes,et al.  Salt-soluble elastin from lathyritic chicks. , 1974, The Biochemical journal.

[31]  B. Sykes,et al.  Isolation of salt-soluble elastin from ligamentum nuchae of copper-deficient calf. , 1974, The Biochemical journal.

[32]  R. Stroud.  A family of protein-cutting proteins. , 1974, Scientific American.

[33]  K. Ohlsson,et al.  The Neutral Proteases of Human Granulocytes , 1974 .

[34]  N. Ackerman,et al.  Release of Lysosomal Enzymes by Alveolar Mononuclear Cells , 1974, Nature.

[35]  L. Sandberg,et al.  Molecular Model for Elastin Structure and Function , 1973, Nature.

[36]  D. W. Smith,et al.  Biosynthesis of soluble elastin by pig aortic tissue in vitro. , 1973, The Journal of biological chemistry.

[37]  N. Blumenkrantz,et al.  Biosynthesis of collagen in pseudoxanthoma elasticum. , 1973, Acta dermato-venereologica.

[38]  R. Priest,et al.  Collagen of Marfan Syndrome is Abnormally Soluble , 1973, Nature.

[39]  S. Lamberg,et al.  Synthesis and degradation of hyaluronic acid in the cultured fibroblasts of Marfan's disease. , 1973, The Journal of clinical investigation.

[40]  A. Janoff.  Purification of human granulocyte elastase by affinity chromatography. , 1973, Laboratory investigation; a journal of technical methods and pathology.

[41]  K. Ohlsson,et al.  The neutral proteases of human granulocytes. Isolation and partial characterization of two granulocyte collagenases. , 1973, European journal of biochemistry.

[42]  L. Robert,et al.  Studies on a human blood platelet protease with elastolytic activity. , 1973, Biochimica et biophysica acta.

[43]  W R Gray,et al.  Isolation and amino acid sequences of tropoelastin peptides. , 1973, The Journal of biological chemistry.

[44]  R. Ross,et al.  THE ELASTIC FIBER A REVIEW , 1973 .

[45]  D. Varadi,et al.  Studies on the chemistry and fine structure of elastic fibers from normal adult skin. , 1972, The Journal of investigative dermatology.

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

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

[48]  G. Martin,et al.  The production of lysyl oxidase by human fibroblasts in culture. , 1972, Archives of biochemistry and biophysics.

[49]  D. Prockop,et al.  The biosynthesis of collagen. 1. , 1972, The New England journal of medicine.

[50]  R. Ross,et al.  THE SMOOTH MUSCLE CELL I. In Vivo Synthesis of Connective Tissue Proteins , 1971 .

[51]  J. Uitto,et al.  Biochemical characterization of pseudoxanthoma elasticum: collagen biosynthesis in the skin. , 1971, The Journal of investigative dermatology.

[52]  R. Ross,et al.  Elastic fibers in the body. , 1971, Scientific American.

[53]  G. Martin,et al.  Cross-linking of collagen and elastin. Properties of lysyl oxidase. , 1970, Biochemistry.

[54]  D. W. Smith,et al.  The purification and partial characterization of a soluble elastin-like protein from copper-deficient porcine aorta. , 1969, Biochemistry.

[55]  R. Ross,et al.  THE ELASTIC FIBER , 1969, The Journal of cell biology.

[56]  G. Martin,et al.  The cross-linking of collagen and elastin: enzymatic conversion of lysine in peptide linkage to alpha-aminoadipic-delta-semialdehyde (allysine) by an extract from bone. , 1968, Proceedings of the National Academy of Sciences of the United States of America.

[57]  J. Uitto,et al.  Collagen metabolism of the skin in Marfan's syndrome. , 1968, Clinica chimica acta; international journal of clinical chemistry.

[58]  P. Beighton.  X–linked Recessive Inheritance in the Ehlers–Danlos Syndrome* , 1968, British medical journal.

[59]  A. Mehregan.  Elastosis perforans serpiginosa: a review of the literature and report of 11 cases. , 1968, Archives of dermatology.

[60]  K. Hashimoto,et al.  Electron microscopic studies of normal and abnormal elastic fibers of the skin. , 1967, The Journal of investigative dermatology.

[61]  R. Gorlin,et al.  Cutis laxa. A manifestation of generalized elastolysis. , 1965 .

[62]  V. McKusick,et al.  PSEUDOXANTHOMA ELASTICUM: A CLINICAL AND HISTOPATHOLOGICAL STUDY , 1963, Medicine.

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

[64]  J. Edwards,et al.  Pseudoxanthoma elasticum and angioid streaks. A review of 106 cases. , 1961, The American journal of medicine.

[65]  R. Winkelmann,et al.  Elastosis Perforans (Perforating Elastosis) , 1960 .

[66]  A. Cohen,et al.  Electron microscopic observations on the lesion of elastosis perforans serpiginosa. , 1960, The Journal of investigative dermatology.

[67]  K. Hashimoto,et al.  Elastosis Perforans Serpiginosa , 1960 .

[68]  W. Lobitz,et al.  Pseudoxanthoma elasticum micro-incineration. , 1950, The Journal of investigative dermatology.

[69]  I. Banga,et al.  The elastolytic activity of pancreatic extracts. , 1950, The Biochemical journal.

[70]  P. Livingston.  Role of Heterophoria in Binocular Disharmony , 1937, British medical journal.

[71]  G. M. Fischer,et al.  In vivo effects of sex hormones on aortic elastin and collagen dynamics in castrated and intact male rats. , 1978, Endocrinology.

[72]  S. Gordon,et al.  Evidence for increased protease activity secreted from cultured fibroblasts from patients with pseudoxanthoma elasticum. , 1978, Connective tissue research.

[73]  J. Fierer,et al.  Elastin degradation in human and experimental emphysema. , 1977, Advances in experimental medicine and biology.

[74]  R. Page,et al.  Studies on the action of lysyl oxidase on soluble elastin. , 1977, Advances in experimental medicine and biology.

[75]  W. Hornebeck,et al.  Elastase-like enzymes in aortas and human breast carcinomas: quantitative variations with age and pathology. , 1977, Advances in experimental medicine and biology.

[76]  T. Krieg,et al.  The Marfan’s Syndrome , 1977 .

[77]  R. Mecham,et al.  A high molecular weight species of soluble elastin-proelastin. , 1977, Advances in experimental medicine and biology.

[78]  J. Rosenbloom,et al.  Evidence that tropoelastin exists as a random coil. , 1977, Advances in experimental medicine and biology.

[79]  G. Gerber,et al.  Studies on cross-linked regions of elastin. , 1977, Advances in experimental medicine and biology.

[80]  T. Krieg,et al.  The marfan's syndrome. In vitro study of collagen metabolism in tissue specimens of the aorta. , 1977, Experimental cell biology.

[81]  R. Ross,et al.  The morphogenesis of elastic fibers. , 1977, Advances in experimental medicine and biology.

[82]  P. Byers,et al.  An X-linked form of cutis laxa due to deficiency of lysyl oxidase. , 1976, Birth defects original article series.

[83]  L B Sandberg,et al.  Elastin structure in health and disease. , 1976, International review of connective tissue research.

[84]  J. Uitto,et al.  Intracellular Steps in the Biosynthesis of Collagen , 1976 .

[85]  R. Senior,et al.  Comparison of the elastolytic effects of human leukocyte elastase and porcine pancreatic elastase. , 1976, Biochemical and biophysical research communications.

[86]  R. Leachman,et al.  Lysyl oxidase deficiency in Ehlers-Danlos syndrome type V. , 1975, Connective tissue research.

[87]  Victor A. McKusick,et al.  Heritable disorders of connective tissue , 1972 .

[88]  D. Jackson,et al.  Carbohydrate content of insoluble elastins prepared from adult bovine and calf ligamentum nuchae and tropoelastin isolated from copper-deficient porcine aorta. , 1971, The Biochemical journal.

[89]  H. E. Christensen,et al.  Pseudoxanthoma elasticum. A clinico-pathological study. , 1970, Acta dermato-venereologica.

[90]  W. Carnes,et al.  Copper and connective tissue metabolism. , 1968, International review of connective tissue research.

[91]  J. Parker,et al.  Ultrastructural changes of elastic fibers in pseudoxanthoma elasticum. A study of histogenesis. , 1967, Archives of pathology.