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Glycine cleavage system
Enzymes that break down glycine / From Wikipedia, the free encyclopedia
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The glycine cleavage system (GCS) is also known as the glycine decarboxylase complex or GDC. The system is a series of enzymes that are triggered in response to high concentrations of the amino acid glycine.[1] The same set of enzymes is sometimes referred to as glycine synthase when it runs in the reverse direction to form glycine.[2] The glycine cleavage system is composed of four proteins: the T-protein, P-protein, L-protein, and H-protein. They do not form a stable complex,[3] so it is more appropriate to call it a "system" instead of a "complex". The H-protein is responsible for interacting with the three other proteins and acts as a shuttle for some of the intermediate products in glycine decarboxylation.[2] In both animals and plants, the glycine cleavage system is loosely attached to the inner membrane of the mitochondria. Mutations in this enzymatic system are linked with glycine encephalopathy.[2]
Glycine cleavage H-protein | |||||||||
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![]() refined structures at 2 angstroms and 2.2 angstroms of the two forms of the h-protein, a lipoamide-containing protein of the glycine decarboxylase | |||||||||
Identifiers | |||||||||
Symbol | GCV_H | ||||||||
Pfam | PF01597 | ||||||||
Pfam clan | CL0105 | ||||||||
InterPro | IPR002930 | ||||||||
SCOP2 | 1htp / SCOPe / SUPFAM | ||||||||
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Glycine cleavage T-protein, Aminomethyltransferase folate-binding domain | |||||||||
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![]() crystal structure of a component of glycine cleavage system: t-protein from pyrococcus horikoshii ot3 at 1.5 a resolution | |||||||||
Identifiers | |||||||||
Symbol | GCV_T | ||||||||
Pfam | PF01571 | ||||||||
Pfam clan | CL0289 | ||||||||
InterPro | IPR006222 | ||||||||
SCOP2 | 1pj5 / SCOPe / SUPFAM | ||||||||
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Glycine cleavage T-protein C-terminal barrel domain | |||||||||
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![]() crystal structure of t-protein of the glycine cleavage system | |||||||||
Identifiers | |||||||||
Symbol | GCV_T_C | ||||||||
Pfam | PF08669 | ||||||||
InterPro | IPR013977 | ||||||||
SCOP2 | 1pj5 / SCOPe / SUPFAM | ||||||||
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