Abstract
This chapter is a review of present biochemical knowledge of those abnormalities of the metabolism of sulfur-containing amino acids which are associated with disorders of the central nervous system. The reader is referred to Vol. III, Chapter 8, of the Handbook for fuller discussion of the normal metabolism of these compounds in brain. However, attention will be called to gaps in our knowledge of normal metabolism which the study of these metabolic lusus naturae has brought to light.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
Similar content being viewed by others
References
C. M. B. Field, N. A. J. Carson, D. C. Cusworth, C. E. Dent, and D. W. Neill, Homo-cystinuria: A new disorder of metabolism, Abstr. Xth Internat. Congr. Ped., Lisbon, p. 274 (1962).
N. A. J. Carson, D. C. Cusworth, C. E. Dent, C. M. B. Field, D. W. Neill, and R. G. Westall, Homocystinuria: A new inborn error of metabolism associated with mental deficiency, Arch. Dis. Child. 38: 425 (1963).
T. Gerritsen, J. G. Vaughn, and H. A. Waisman, The identification of homocystine in the urine, Biochem. Biophy. Res. Comm., 9: 493 (1962).
T. Gerritsen and H. A. Waisman, Homocystinuria, an error in the metabolism of methionine, Ped. 33: 413 (1964).
G. E. Gaull, N. A. J. Carson, C. E. Dent, and C. M. B. Field, Homocystinuria: Clinical and pathological description of 10 cases, Proc. Internat. Congr. on the Scient. Study of Mental Retard., Copenhagen, p. 91 (1964).
N. A. J. Carson, C. E. Dent, C. M. B. Field, and G. E. Gaull, Homocystinuria: Clinical and pathological review of ten cases, J. Ped. 66: 565 (1965).
R. N. Schimke, V. A. McKusick, T. Huang, and A. D. Pollack, Homocystinuria. Studies of 20 families with 38 affected members, J. Amer. Med. Assn., 193: 87 (1965).
J. B. Gibson, N. A. J. Carson, and D. W. Neill, Pathological findings in homocystinuria, J. Clin. Path. 17, 427 (1964).
H. H. White, L. P. Rowland, S. Araki, H. L. Thompson, and D. Cowen, Homocystinuria, Arch. Neurol. 13: 455(1965).
G. W. Komrower and V. K. Wilson, Homocystinuria, Proc. Roy. Soc. Med. 56: 996 (1963).
J. D. Finkelstein, S. H. Mudd, F. Irreverre, and L. Laster, Homocystinuria due to cystathionine synthetase deficiency: The mode of inheritance, Science 146: 785 (1964).
G. L. Spaeth and G. W. Barber, Homocystinuria in a mentally retarded child and her normal cousin, Trans. Amer. Acad. Ophthal. and Otolarng. 69: 912 (1965).
C. Kennedy, V. E. Shih, and L. P. Rowland, Homocystinuria: A report in two siblings, Pediatrics 36: 736 (1965).
E. A. Werder, H. Curtius, P. W. Anders, and A. Prader, Homocystinuria, Helvetica Paediatrica Acta 21: 1 (1966).
H. G. Dunn, T. L. Perry, and C. L. Dolman, Homocystinuria, Neurology 16: 407 (1966).
E. M. Brett, Homocystinuria with epilepsy, Proc. Roy. Soc. Med. 59: 484 (1966).
M. C. Carey, D. E. Donovan, O. FitzGerald, and F. D. McAuley, Homocystinuria. I. A. clinical and pathological study of nine subjects in six families, Amer. J. Med. 45: 7 (1968).
B. Gaudier, P. Francois, G. Biserte, M. Dautrevaux, J. P. Nuyts, and E. Bombart, L’Homo-cystinuria (A propos de trois observations), Arch. Franc. Ped. pp. 25, 541 (1968).
H. R. Spiro, R. N. Schimke, and J. P. Welch, Schizophrenia in a patient with a defect in methionine metabolism. J. Nervous Mental Dis. 141: 285 (1965).
S. H. Mudd, J. D. Finkelstein, F. Irreverre, and L. Laster, Homocystinuria: an enzymatic defect, Science 143, 1443 (1964).
S. H. Mudd, J. D. Finkelstein, F. Irreverre, and L. Laster, Transsulfuration in mammals. Microassays and tissue distributions of three enzymes of the pathway, J. Biol. Chem. 240: 382(1965).
D. P. Brenton, D. C. Cusworth, and G. E. Gaull, Homocystinuria. Some biochemical studies, Proc. Internat. Congr. on the Scient. Study of Mental Retard., Copenhagen, p. 79 (1964).
D. P. Breton, D. C. Cusworth, and G. E. Gaull, Homocystinuria. Biochemical studies of tissues including a comparison with cystathionuria, Ped. 35: 50 (1965).
T. Gerritsen and H. A. Waisman, Homocystinuria: Absence of cystathionine in the brain, Science 145:588 (1964).
S. H. Mudd, L. Laster, J. D. Finkelstein, and F. Irreverre, in Amines and Schizophrenia (H. E. Himwith, S. S. Kety, and J. R. Smythies, eds.) p. 247, Pergamon Press, Oxford and New York (1966).
G. E. Gaull and M. K. Gaitonde, Homocystinuria: An observation on the inheritance of cystathionine synthase deficiency. J. Med. Genet. 3: 194 (1966).
B. W. Uhlendorf and S. H. Mudd, Cystathionine synthase in tissue culture derived from human skin: enzyme defect in homocystinuria, Science 160: 1007 (1968).
S. H. Mudd, J. D. Finkelstein, F. Irreverre, and L. Laster, Threonine dehydratase activity in humans lacking cystathionine synthase, Biochem. Biophys. Res. Commun. 19: 565 (1965).
F. C. Brown, J. Mallady, and J. A. Roszell, The separation of rat liver serine dehydratase and cystathionine synthase, J. Biol. Chem. 241: 5220 (1966).
H. Nakagawa and H. Kimura, Purification and properties of cystathionine synthetase from rat liver: Separation of cystathionine synthetase from serine dehydratase, Biochem. Biophys. Res. Commun. 32: 208 (1968).
G. E. Gaull, D. C. Cusworth, D. Breton, and C. E. Dent, The biochemical defect in homocystinuria, Am. Ped. Soc, Abstr. 25 (1964).
D. P. Breton, D. C. Cusworth, and G. E. Gaull, Homocystinuria: Metabolic studies on three patients, J. Ped. 67: 58 (1965).
W. K. Hall, M. E. Coryell, J. G. Hollowell, and T. G. Thevaos, A metabolic study of homocystinuria, Fed. Proc. 24: 470 (1965).
T. L. Perry, S. Hansen, H. Bar, and L. MacDougall, Homocystinuria: Excretion of a new sulfur-containing amino acid in urine, Science 152: 776 (1966).
T. L. Perry, S. Hansen, and L. MacDougall, Homolanthionine excretion in homocystinuria, Science 152: 1750 (1966).
T. L. Perry, S. Hansen, L. MacDougall, and P. D. Warrington, Sulfur-containing amino acids in the plasma and urine of homocystinuria, Clin. Chim. Acta 15: 409 (1967).
L. Laster, S. H. Mudd, J. D. Finkelstein, and E. Irreverre, Homocystinuria due to cystathionine synthase deficiency: the metabolism of L-methionine, J. Clin. Invest. 44: 1708 (1965).
L. Laster, G. L. Spaeth, S. H. Mudd, and J. D. Finkelstein, Homocystinuria due to cystathionine synthase deficiency, Ann. Int. Med. 63: 1117 (1965).
W. C. Rose and R. L. Wixom, Amino acid requirements of man: XIII, Sparing effect of cystine on the methionine requirement, J. Biol. Chem. 216: 95 (1955).
D. P. Brenton, D. C. Cusworth, C. E. Dent, and E. E. Jones, Homocystinuria. Clinical and dietary studies, Quart. J. Med. 35: 325 (1966).
G. E. Gaull, The pathogenesis of homocystinuria. Implications for treatment, Am. J. Dis. Child. 113: 103(1967).
D. C. Cusworth and C. E. Dent, Homocystinuria, Brit. Med. Bull. 25: 42 (1969).
D. P. Brenton and D. C. Cusworth, Homocystinuria: Metabolism of 35(S)-methionine, Clin. Sci. 31: 197(1966).
E. D. Harris, Jr. and A. Sjoerdsma, Collagen profile in various clinical conditions, Lancet 2: 7466(1966).
P. Henkind and N. Ashton, Ocular pathology in homocystinuria, Trans. Ophthal. Soc. U.K. 85: 21 (1965).
M. C. Carey, J. J. Fennelly, and O. Fitzgerald, Homocystinuria II. Subnormal serum folate levels, increased folate clearance and effects of folic acid therapy, Am. J. Med. 45: 26 (1968).
G. Gaull, in Brain Damage in Inborn Errors of Metabolism (E. van Praag, ed.), p. 67, Bohn Publ., Haarlem (1968).
J. A. Stekol and J. Szaran, Pathological effects of excessive methionine in the diet of growing rats, J. Nutrit. 77: 81 (1962).
J. V. Klavins, Pathology of amino acid excess-1. Effects of administration of excessive amounts of sulphur-containing amino acids; homocystine, Brit. J. Exp. Path. 44: 507 (1963).
J. Carbon and H. David, Studies on the thionucleotides in transfer ribonucleic acid. Addition of Af-ethylmaleimide and formation of mixed disulfides with thiol compounds, Biochemistry 7: 3851 (1968).
S. R. Mardashev, in Proc. of the Symposium on Chemical and Biological Aspects of Pyridoxal Catalysis (E. Snell ed.), p. 227, Pergamon Press, London (1963).
H. H. Tallan, S. Moore, and W. H. Stein, L-Cystathionine in human brain, J. Biol. Chem. 230, 707 (1958).
G. E. Gaull and M. K. Gaitonde, A study of sulphur amino acids of rat tissues, Biochem. J. 102: 294(1967).
M. K. Gaitonde and G. E. Gaull, A procedure for quantitative analysis of the sulphur amino acids of rat tissues, Biochem. J. 102: 959 (1967).
B. Lombardi, Pathogenesis of fatty liver, Fed. Proc. 24: 1200 (1965).
G. De La Haba and H. Holtzer, Chondroitin sulfate: Inhibition of synthesis by puromycin, Science 149: 1273 (1965).
F. G. Michels and J. T. Smith, A comparison of the utilization of organic and inorganic sulfur by the rat, J. Nutrit. 87: 217 (1965).
H. Sprince, C. M. Parker, J. A. Josephs, and C. R. Wilkizeski, Convulsant activity of homocysteine in the rat: Protection by homoserine, betaine, and glycine, Fed. Proc. 27: 652 (1968).
G. M. Komrower, A. M. Lambert, D. C. Cusworth, and R. G. Westall, Dietary treatment of homocystinuria, Arch. Dis. Child. 41: 666 (1966).
T. L. Perry, H. G. Dunn, S. Hansen, L. MacDougall, and P. D. Warrington, Early diagnosis and treatment of homocystinuria, Pediatrics 37: 502 (1966).
I. B. Sardharwalla, S. H. Jackson, H. D. Hawke, and A. Sass-Kortsak, Homocystinuria: A study with low-methionine diet in three patients, Canad. Med. Assn. J. 99, 731 (1968).
B. Hagberg and L. Hambraeus, Some aspects of the diagnosis and treatment of homocystinuria, Dev. Med. and Child Neurol. 10: 479 (1968).
T. L. Perry, D. L. Love, S. H. Hansen, L. E. Crawford, and B. Tischler, Treatment of homocystinuria with a low-methionine diet, supplemental cystine, and a methyl donor, Lancet 1: 474(1968).
G. W. Barberand G. L. Spaeth, Pyridoxine therapy in homocystinuria, Lancet 1: 337 (1967).
C. Hooft, D. Carton, and W. Samyn, Pyridoxine treatment in homocystinuria, Lancet 1: 1384(1967).
B. Turner, Pyridoxine treatment in homocystinuria, Lancet 2: 1151 (1967).
G. E. Gaull, D. K. Rassin, and J. A. Sturman, Pyridoxine-dependency in homocystinuria, Lancet 2: 1302 (1968).
G. E. Gaull, J. A. Sturman, and D. K. Rassin, Enzymatic and metabolic studies of homocystinuria: Effects of pyridoxine, Neuropadiatrie 1: 199 (1969).
D. C. Cusworth and C. E. Dent, Homocystinuria, Brit. Med. Bull. 25: 42 (1969).
L. Lambraeus, L. Wranne, and R. Lorentsson, Biochemical and thereapeutic studies in two cases of homocystinura, Clin. Sci. 35: 757 (1968).
J. G. Hallowell, Jr., M. E. Corell, W. K. Hall, J. F. Findley, and T. G. Thevaos, Homocystinuria as affected by pyridoxine, folic acid and vitamin B12, Proc. Soc. Exp. Biol. Med. 129: 327 (1968).
T. Yoshida, K. Tada, Y. Yokoyoma, and T. Arakawa, Homocystinuria of vitamin B6 dependent type, Tohoku J. Exp. Med. 96: 235 (1968).
P. W. K. Wong, V. Schwarz, and G. M. Komrower, The biosynthesis of cystathionine in patients with homocystinuria, Pediat. Res. 2: 149 (1968).
H. Harris, L. S. Penrose, and D. H. H. Thomas, Cystathioninuria, Am. Human Genet. 23: 442(1959).
G. W. Frimpter, A. Haymovitz, and M. Horwith, Cystathioninuria, New Engl. J. Med. 268: 333 (1963).
J. Mongeau, M. Hilgartner, H. G. Worthen, and G. W. Frimpter, Cystathioninuria: Study of an infant with normal mentality, thrombocytopenia, and renal calculi, J. Pediat. 69: 1113 (1966).
S. Berlow, Studies in cystathioninuria, Am. J. Dis. Child. 112, 135 (1966).
K. N. Shaw, E. Lieberman, R. Koch, and G. N. Donnell, Cystathioninuria, Am. J. Dis. Child 113: 119 (1967).
H. Haraguchi, E. Iwatani, F. Yamashita, and T. Nagayama, Studies on cystathioninuria, Igakundayumi (Jap.) 61: 72 (1967).
K. Tada, T. Yoshida, Y. Yokoyama, T. Sato, H. Nakagawa, and T. Arakawa, Cystathioninuria not associated with vitamin B6 dependency: A probably new type of cystathioninuria, Tohoku J. Exp. Med. 95: 235 (1968).
T. L. Perry, G. C. Robinson, J. M. Teasdale, and S. Hansen, Concurrence of cystathioninuria, nephrogenic diabetes insipidus and severe anemia, New Eng. J. Med. 276: 721 (1967).
T. L. Perry, D. F. Hard wick, S. Hansen, D. L. Love, and S. Israels, Cystathioninuria in two healthy siblings, New Engl. J. Med. 278: 590 (1968).
D. T. Whelan and C. R. Scriver, Cystathioninuria and renal iminoglycinuria in a pedigree, New Engl. J. Med. 278: 924 (1968).
C. Hooft and D. Carton, Cystathioninemia in three siblings, Abstr. Soc. for Study Inborn Errors of Metab., Zurich (1968).
K. N. F. Shaw, E. Lieberman, G. N. Donnell, R. Koch, M. L. Weil, and J. H. Menkes, Cystathioninuria, Abstr. XII Internat. Cong. Ped., Mexico City, Vol. III, p. 410 (1968).
G. W. Frimpter, Cystathioninuria: Nature of the defect, Science 149: 1095 (1965).
J. D. Finkelstein, S. H. Mudd, F. Irreverre, and L. Laster, Deficiencies of cystathionase and homoserine dehydratase activities in homocystinuria, Proc. Nat. Acad. Sci. 55: 865 (1966).
T. L. Perry, S. Hansen, D. Love, and C. A. Finch, iV-acetylcystathioninuria, Nature 219: 178(1968).
G. W. Frimpter and A. J. Greenberg, Renal clearance of cystathionine in homozygous and heterozygous cystathioninuria, cystinura, and the normal state. J. Clin. Investigation 46: 975(1967).
D. B. Hope, Cystathionine accumulation in the brains of pyridoxine-deficient rats, J. Neurochem. 11: 327 (1964).
D. B. Hope, Studies of taurine and cystathionine in brain, Fourth Internat. Congr. of Biochem., Colloquia XIII, p. 63, Pergamon Press, New York (1958).
M. E. Swendseid, J. Villalobos, and B. Friedrich, Free amino acids in plasma and tissues of rats fed a vitamin B6-deficient diet, J. Nutrit. 82: 206 (1964).
J. K. Tews and R. A. Lovell, The effect of a nutritional pyridoxine deficiency on free amino acids and related substances in mouse brain, J. Neurochem. 14: 1 (1967).
R. S. Harding, J. E. Canham, and H. E. Säuberlich, in Technicon Symposium on Automated Analytical Chemistry, New York (1965).
P. Fourman, J. W. Summerscales, and D. M. Morgan, Cystathioninuria from pyridoxine deficiency complicating treatment of hypercalcaemia in a cretin, Arch. Dis. Childh. 41: 273 (1966).
A. Baysal, Experimental vitamin B6 depletion in man, J. Nutrit. 89: 19 (1966).
J. A. Sturman, P. A. Cohen, and G. E. Gaull, Effects of deficiency of vitamin B6 on transulfuration, Biochem. Med. 3: 244 (1969).
L. R. Gjessing, Cystathioninuria and vanillacticaciduria in neuroblastoma and argen-taffinoma, Lancet 2: 1281 (1963).
L. R. Gjessing, Studies of functional neural tumors. II. Cystathioninuria, Scand. J. Clin. Lab. Invest. 15: 474 (1963).
L. R. Gjessing, Studies of functional neural tumors, III. Cystathionine in tumor tissue, Scand. J. Clin. Lab. Invest. 15: 479 (1963).
E. Lieberman, K. N. F. Shaw, and G. N. Donnell, Cystathioninuria in galactosemia and certain types of liver disease, Pediatrics 40: 828 (1967).
J. Sturman, D. Rassin, and G. Gaull, Trans-sulfuration enzymes and accumulation of cystathionine in brain, Fed. Proc. 27: 614 (1968).
L. R. Gjessing and K. Mauritzen, Cystathioninuria in hepatoblastoma, Scand. J. Clin. Lab. Invest. 17: 513(1965).
P. A. Voute, Jr. and S. K. Wadman, Cystathioninuria in hepatoblastoma, Clin. Chim. Acta 22: 373 (1968).
L. R. Gjessing, Cystathioninuria during a load of thyroxine, Scand. J. Clin. Lab. Invest. 16: 680(1964).
C. Ressler, J. Nelson, and M. Pfeffer, A pyridoxal-beta-cyanoalanine relation in the rat, Nature 203: 1286 (1964).
M. Pfeffer and C. Ressler, Beta-cyanoalanine inhibitor of rat liver cystathionase, Biochem. Pharmacol. 16, 2299 (1967).
L. Laster, F. Irreverre, S. H. Mudd, and W. D. Heizer, A previously unrecognized disorder of metabolism of sulfur-containing compounds. Abnormal urinary excretion of S-sulfo-L-cysteine, sulfite, and thiosulfate in a severely retarded child with ectopia lentis, J. Clin. Invest. 46: 1082(1967).
F. Irreverre, S. H. Mudd, W. D. Heizer, and L. Laster, Sulfite oxidase deficiency: Studies of a patient with mental retardation, dislocated ocular lenses, and abnormal urinary excretion of S-sulfo-L-cysteine, sulfite, and thiosulfate, Biochem. Med. 1: 187 (1967).
S. A. Mudd, F. Irreverre, and L. Laster, Sulfite oxidase deficiency in man: demonstration of the enzymatic defect, Science 156: 1599 (1967).
M. G. Ampola, M. L. Efron, E. M. Bixby, and E. Meshorer, Mental deficiency and a new aminoaciduria, Am. J. Dis. Child. 117: 66 (1969).
J. C. Crawhall, R. Parker, W. Sneddon, and E. P. Young, Beta-mercaptolactate-cysteine disulfide in the urine of a mentally retarded patient, Am. J. Dis. Child. 117: 71 (1969).
W. Pollin, P. V. Cardon, Jr., and S. S. Kety, Effects of amino acid feedings in patients treated with iproniazid, Science 133: 104 (1961).
L. C. Park, R. J. Baldessarini, and S. S. Kety, Methionine effects on chronic schizophrenics, Arch. Gen. Psychiat. 12: 346(1965).
A. Agur and A. Pinsky, Comparative study of taurine, sulphur-containing amino acids and phospholipids in schizophrenic and normal blood and urine, Israel J. Chem. 2: 318 (1969).
C. Hooft, J. Timmermans, A. Snoeck, I. Aubener, W. Oyaert, and C. Van Den Heude, Syndrome de malabsorption de 1a methionine, Ann. Paediat. (Basel) 73: 205 (1965).
L. Reiner, F. Misani, and P. Weiss, Studies on nitrogen trichloride treated prolamines. VI. Suppression of the development of convulsions with methionine, Arch. Biochem. 25: 447 (1950).
O. E. Sellinger, J. M. Azcurra, and W. G. Ohlsson, Methionine, sulfoximine seizures, VIII, The dissociation of the convulsant and glutamine synthetase inhibitory effects, J. Pharm. Exper. Therap. 164: 212 (1968).
H. O. Goodman, J. S. King, and J. J. Thomas, Urinary excretion of beta-amino isobutyric acid and taurine in mongolism, Nature 204: 650 (1964).
H. L. Levy, V. E. Shih, P. M. Madigan, V. Karolkewicz, J. R. Carr, A. Lum, A. A. Richards, J. D. Crawford, and R. A. MacCready, Hypermethioninemia with other hyperamino-acidemias, Am. J. Child. 117: 96 (1969).
L. R. Gjessing and S. Halvorsen, Hypermethioninemia in acute tyrosinosis, Lancet 2: 1–132 (1965).
C. R. Scriver, C. L. Clow, and M. Silverberg, Hypermethioninemia in acute tyrosinosis, Lancet 2: 153 (1966).
J. Gentz, R. Jagenburg, and R. Zetterstrom, Tyrosinemia. An inborn error of tyrosine metabolism with cirrhosis of the liver and multiple renal tubular defects, J. Pediat. 66: 670 (1965).
Taniguchi and L. R. Gjessing, Studies on tyrosinosis: 2, Activity of the transaminase, parahydroxyphenylpyruvate oxidase and homogentisic-acid oxidase, Brit. Med. J. 1: 968 (1965).
L. R. Gjessing (ed.), Symposium on Tyrosinosis, Oslo Univ. Press, Oslo (1966).
C. R. Scriver, M. Partington, and A. Sass-Kortsak (eds.), Conference on Hereditary Tyrosinemia, Canad. Med. Assoc. J. 97: 1045 (1967).
G. E. Gaull, D. K. Rassin, and J. A. Sturman, Significance of hypermethioninemia in acute tyrosinosis, Lancet 1: 1318 (1968).
G. E. Gaull, R. C. Harris, and G. Solomon, Tyrosinosis: Biochemical and dietary studies, Pediat. Res. 2: 403 (1968).
G. Bodegard, J. Gentz, B. Lindblad, S. Lindstedt, and R. Zetterstrom, Hereditary tyrosinemia. III. On the differential diagnosis and lack of effect of early dietary treatment, Acta Pediat. Scand. 58: 37 (1969).
H. L. Levy, S. H. Mudd, J. D. Schulman, P. M. Dreyfus, and R. H. Abeles, A derangement in B12 metabolism associated with homocystinemia, cystathioninemia, hypomethionin-emia and methylmalonic aciduria, Am. J. Med. 48: 390 (1970).
S. H. Mudd, H. L. Levy, and R. H. Abeles, A derangement in B12 metabolism leading to homocystinemia, cystathioninemia and methylmalonic aciduria, Biochem. Biophys. Res. Commun. 35: 121 (1969). 190
S. H. Mudd, H. L. Levy, and G. Morrow, Deranged B12 metabolism: Effects on sulfur amino acid metabolism, Biochem. Med. 4: 193 (1970).
S. H. Mudd, B. W. Uhlendorf, K. R. Hinds, and H. L. Levy, Deranged B12 metabolism: Studies of fibroblasts grown in tissue culture, Biochem. Med. 4: 215 (1970).
S. I. Goodman, P. G. Moe, K. B. Hammond, S. H. Mudd, and W. B. Uhlendorf, Homo-cystinuria with methylmalonic aciduria: Two cases in a sibship, Biochem. Med. 4: 500 (1970).
G. E. Gaull, Y. Wada, K. Schneidman, D. K. Rassin, H. H. Tallan, and J. A. Sturman, Homocystinuria: Observations on the biosynthesis of cystathionine and homolanthionine, Pediat. Res. 5: 265 (1971).
H. H. Tallan, T. A. Pascal, K. Schneidman, B. M. Gillam, and G. E. Gaull, Homolanthionine synthesis by human liver cystathionase, Biochem. Biophys. Res. Com. 43: 303 (1971).
G. E. Gaull and F. Schaffner, Electron microscopic changes in hepatocytes of patients with homocystinuria, Pediat. Res. 5: 23 (1971).
K. McCully, Vascular pathology of homocysteinemia: Implications for the pathogenesis of arteriosclerosis, Am. J. Pathol. 56: 111 (1969).
K. McCully, Importance of homocysteine-induced abnormalities of proteoglycan structure in arteriosclerosis, Am. J. Pathol. 59: 181 (1970).
K. McCully and B. Ragsdale, Production of arteriosclerosis by homocysteinemia, Am. J. Pathol. 61: 1 (1970).
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1972 Plenum Press, New York
About this chapter
Cite this chapter
Gaull, G.E. (1972). Abnormal Metabolism of Sulfur-Containing Amino Acids Associated with Brain Dysfunction. In: Lajtha, A. (eds) Handbook of Neurochemistry. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-7172-8_9
Download citation
DOI: https://doi.org/10.1007/978-1-4615-7172-8_9
Publisher Name: Springer, Boston, MA
Print ISBN: 978-1-4615-7174-2
Online ISBN: 978-1-4615-7172-8
eBook Packages: Springer Book Archive