Reactome | AMT transfers NH2CH2 from GCSH:SAMDLL to THF

AMT transfers NH2CH2 from GCSH:SAMDLL to THF

Stable Identifier

R-HSA-5693977

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

(6S)-5,6,7,8-tetrahydrofolate + N(6)-((R)-S(8)-aminomethyldihydrolipoyl)-L-lysyl-(protein) => (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + N(6)-((R)-dihydrolipoyl)-L-lysyl-(protein) + NH4(+), AMT degrades GCSH:SAMDLL to GCSH:DHLL

ReviewStatus

5/5

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AMT transfers NH2CH2 from GCSH:SAMDLL to THF

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The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

The major degradative pathway for the amino acid glycine is via the glycine cleavage system. In the second reaction in this system, the decarboxylated moiety from glycine decarboxylation attached to H protein (GCSH:SAMDLL) is further degraded by mitochondrial aminomethyltransferase (AMT, GCST, T protein) to ammonia (NH3) and GCSH with reduced lipoate. Tetrahydrofolate (THF) is required for this reaction and accepts the methyl group to form 5,10MTHF (Fujiwara et al. 1984).

Literature References

PubMed ID	Title	Journal	Year
16051266	Crystal structure of human T-protein of glycine cleavage system at 2.0 A resolution and its implication for understanding non-ketotic hyperglycinemia Okamura-Ikeda, K, Hosaka, H, Yoshimura, M, Yamashita, E, Toma, S, Nakagawa, A, Fujiwara, K, Motokawa, Y, Taniguchi, H	J Mol Biol	2005
6469978	Mechanism of the glycine cleavage reaction. Further characterization of the intermediate attached to H-protein and of the reaction catalyzed by T-protein Fujiwara, K, Okamura-Ikeda, K, Motokawa, Y	J. Biol. Chem.	1984