Gene name: GCSH

Uniprot entry:

P23434

Protein names:

Glycine cleavage system H protein, mitochondrial (Lipoic acid-containing protein)

Protein sequence:

1_MALRV 6_ VRSVR 11_ ALLCT 16_ LRAVP 21_ SPAAP 26_ CPPRP 31_ WQLGV 36_ GAVRT 41_ LRTGP 46_ ALLSV 51_ RKFTE 56_ KHEWV 61_ TTENG 66_ IGTVG 71_ ISNFA 76_ QEALG 81_ DVVYC 86_ SLPEV 91_ GTKLN 96_ KQDEF 101_ GALES 106_ VKAAS 111_ ELYSP 116_ LSGEV 121_ TEINE 126_ ALAEN 131_ PGLVN 136_ KSCYE 141_ DGWLI 146_ KMTLS 151_ NPSEL 156_ DELMS 161_ EEAYE 166_KYIKS

Protein annotations

Protein functions:

1: The glycine cleavage system catalyzes the degradation of glycine. The H protein (GCSH) shuttles the methylamine group of glycine from the P protein (GLDC) to the T protein (GCST). Has a pivotal role in the lipoylation of enzymes involved in cellular energetics such as the mitochondrial dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex (DLAT), and the mitochondrial dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex (DLST) (PubMed:36190515)