PXLP-K754-GLDC dimer decarboxylates Gly

Stable Identifier
R-HSA-5693967
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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The simplest amino acid, glycine, is catabolised by several different pathways. The major pathway is via the glycine cleavage system. In the first reaction, glycine (Gly) is decarboxylated to carbon dioxide (CO2) and aminomethyl group (NH2CH2) by mitochondrial glycine dehydrogenase (decarboxylating) (GLDC, P protein), a dimeric protein using pyridoxal 5-phosphate (PXPL) as cofactor per subunit (Kume et al. 1991). Mitochondrial glycine cleavage system H protein (GCSH) is used as a co-substrate in this reaction. GCSH uses lipoate as a cofactor which accepts the aminomethylgroup from glycine decarboxylation to form a S-aminomethyldihydrolipoylated protein (GCSH:SAMDLL) (Fujiwara et al. 1991, Fujiwara et al. 1991).

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Catalyst Activity
Catalyst Activity
Title
glycine dehydrogenase (decarboxylating) activity of PXLP-K754-GLDC dimer [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events
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