Reactome | PXLP-K754-GLDC dimer decarboxylates Gly

PXLP-K754-GLDC dimer decarboxylates Gly

Stable Identifier

R-HSA-5693967

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

ReviewStatus

5/5

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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).

Literature References

PubMed ID	Title	Journal	Year
1959641	Lipoylation of H-protein of the glycine cleavage system. The effect of site-directed mutagenesis of amino acid residues around the lipoyllysine residue on the lipoate attachment Motokawa, Y, Fujiwara, K, Okamura-Ikeda, K	FEBS Lett.	1991
1993704	The glycine cleavage system. Molecular cloning of the chicken and human glycine decarboxylase cDNAs and some characteristics involved in the deduced protein structures Koyata, H, Kure, S, Ishiguro, Y, Kume, A, Hiraga, K, Sakakibara, T	J. Biol. Chem.	1991
2025283	The primary structure of human H-protein of the glycine cleavage system deduced by cDNA cloning Motokawa, Y, Fujiwara, K, Okamura-Ikeda, K, Hayasaka, K	Biochem. Biophys. Res. Commun.	1991