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Theγ-Glutamyl Cycle: Diseases Associated with Specific Enzyme Deficiencies

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▸Requests for reprints should be addressed to Alton Meister, M.D., Department of Biochemistry, Cornell University Medical College, 1300 York Ave., New York, NY 10021.

New York, New York

Ann Intern Med. 1974;81(2):247-253. doi:10.7326/0003-4819-81-2-247
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The γ-glutamyl cycle consists of six enzyme-catalyzed reactions that account for the synthesis and degradation of glutathione, a tripeptide that is found in virtually all living cells. It has been proposed that the γ-glutamyl cycle functions in the transport of amino acids across cell membranes. The status of research on the γ-glutamyl cycle is considered. There is evidence that the cycle functions in the kidney and in other tissues. Patients with diseases associated with a deficiency of a specific enzyme of the γ-glutamyl cycle have been found, and the nature of the biochemical defects has been explored.





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