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Glycine cleavage system protein H (aminomethyl carrier)
Symbols GCSH ; GCE; NKH
External IDs OMIM238330 MGI1915383 HomoloGene90880 GeneCards: GCSH Gene
Species Human Mouse
Entrez 2653 68133
Ensembl ENSG00000140905 ENSMUSG00000034424
UniProt P23434 Q91WK5
RefSeq (mRNA) NM_004483 NM_026572
RefSeq (protein) NP_004474 NP_080848
Location (UCSC) Chr 16:
81.12 – 81.13 Mb
Chr 8:
116.98 – 116.99 Mb
PubMed search [1] [2]

Glycine cleavage system H protein, mitochondrial is a protein that in humans is encoded by the GCSH gene.[1][2][3]


The enzyme system for cleavage of glycine (glycine cleavage system), which is confined to the mitochondria, is composed of 4 protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein; this protein), T protein (a tetrahydrofolate-requiring aminomethyltransferase enzyme), and L protein (a lipoamide dehydrogenase).[3] The H protein shuttles the methylamine group of glycine from the P protein to the T protein.

Clinical significance[edit]

Glycine encephalopathy, also called nonketotic hyperglycinemia (NKH), may be due to a defect in any one of these enzymes.[3]


  1. ^ Koyata H, Hiraga K (Mar 1991). "The glycine cleavage system: structure of a cDNA encoding human H-protein, and partial characterization of its gene in patients with hyperglycinemias". Am J Hum Genet 48 (2): 351–61. PMC 1683031. PMID 1671321. 
  2. ^ Fujiwara K, Okamura-Ikeda K, Hayasaka K, Motokawa Y (Jun 1991). "The primary structure of human H-protein of the glycine cleavage system deduced by cDNA cloning". Biochem Biophys Res Commun 176 (2): 711–6. doi:10.1016/S0006-291X(05)80242-6. PMID 2025283. 
  3. ^ a b c "Entrez Gene: GCSH glycine cleavage system protein H (aminomethyl carrier)". 

Further reading[edit]