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Journal of Nutrition Vol. 94 No. 2 February 1968, pp. 147-150
Copyright © 1968 by American Society for Nutrition
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Derivatives and Analogs of Cysteine and Selected Sulfhydryl Compounds in Nutritional Muscular Dystrophy in Chicks1

J. N. Hathcock2, S. J. Hull and M. L. Scott

Department of Poultry Science and Graduate School of Nutrition, Cornell University, Ithaca, New York

The effects of derivatives and analogs of cysteine and selected sulfhydryl compounds on nutritional muscular dystrophy were studied, using chicks deficient in vitamin E and cystine. The results confirmed earlier indications that mercaptoethylamine is slightly protective against this disorder. Lipoic acid was shown to have a protective effect in a diet marginally deficient but not in a diet severely deficient in sulfur amino acids. Isethionic acid and BAL had no effects on the dystrophy. Of the S-substituted cysteines, S-benzylcysteine accentuated the dystrophy by antagonizing cystine, and did not counteract the effectiveness of vitamin E. S-Carbamylcysteine prevented dystrophy and severely inhibited growth; these effects may have been due to decarbamylation of the compound. S-Methylcysteine and S-ethylcysteine had no effect on dystrophy. The cysteine analog, allylglycine, greatly increased the severity of the dystrophic lesions in the absence but not in the presence of vitamin E. Since the dystrophy-provoking effect of allylglycine was overcome by increased amounts of dietary cystine, these results give additional support to the hypothesis that cysteine is the functional sulfur compound in one of the pathways involved in the prevention of nutritional muscular dystrophy in the chick; vitamin E may be concerned in another pathway.

1 These investigations were supported in part by Public Health Service Research Grant no. NB-05632 from the National Institute of Neurological Diseases and Blindness, and by grants from the Muscular Dystrophy Associations of America, Inc., the Selenium-Tellurium Development Association, Inc., and by Hoffmann-LaRoche, Inc.

2 Present address: Department of Biochemistry, School of Medicine, St. Louis University, St. Louis, Missouri.

Manuscript received 21 September 1967.




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