Glycine recycling
Unless otherwise stated all data on this page refer to the human proteins. Gene information is provided for human (Hs), mouse (Mm) and rat (Rn).
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The glycine cleavage system (a.k.a. the glycine decarboxylase complex) is active at mitochondria. It contains four loosely associated proteins that catalyse the following (reversible) reaction: Glycine + tetrahydrofolate + NAD+ <-> 5,10-methylenetetrahydrofolate + CO2 + NH3 + NADH + H+.
Enzymes
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glycine cleavage system protein H
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Database page citation:
Glycine recycling. Accessed on 11/12/2024. IUPHAR/BPS Guide to PHARMACOLOGY, http://www.guidetopharmacology.org/GRAC/FamilyDisplayForward?familyId=1026.
Concise Guide to PHARMACOLOGY citation:
Alexander SPH, Fabbro D, Kelly E, Mathie AA, Peters JA, Veale EL, Armstrong JF, Faccenda E, Harding SD, Davies JA et al. (2023) The Concise Guide to PHARMACOLOGY 2023/24: Enzymes. Br J Pharmacol. 180 Suppl 2:S289-373.
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Loss of function deletions and mutations in the genes encoding the proteins of the glycine cleavage system are known to cause the elevated levels of glysine that drive glycine encephalopathy (non-ketotic hyperglycinemia; NKH).