Names & Taxonomy

Uniprot ID:
P23434
Entry Name:
GCSH_HUMAN
Status:
reviewed
Protein Names:
Glycine cleavage system H protein, mitochondrial (Lipoic acid-containing protein)
Gene Names:
GCSH
Gene Names Primary:
GCSH
Organism:
Homo sapiens (Human)

Structure

Length:
173
Sequence:
MALRVVRSVRALLCTLRAVPSPAAPCPPRPWQLGVGAVRTLRTGPALLSVRKFTEKHEWVTTENGIGTVGISNFAQEALGDVVYCSLPEVGTKLNKQDEFGALESVKAASELYSPLSGEVTEINEALAENPGLVNKSCYEDGWLIKMTLSNPSELDELMSEEAYEKYIKSIEE
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Mitochondrion.

Function

Function:
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).
Cofactor:
COFACTOR: Name=(R)-lipoate; Xref=ChEBI:CHEBI:83088; ; Note=Binds 1 lipoyl cofactor covalently.;
Cross Reference Drug Bank:
DB00145
Gene Ontology Go:
glycine cleavage complex
mitochondrial matrix
mitochondrion
aminomethyltransferase activity
cellular nitrogen compound metabolic process
glycine catabolic process
glycine decarboxylation via glycine cleavage system
glyoxylate metabolic process
methylation
small molecule metabolic process
Gene Ontology Biological Process:
cellular nitrogen compound metabolic process
glycine catabolic process
glycine decarboxylation via glycine cleavage system
glyoxylate metabolic process
methylation
small molecule metabolic process
Gene Ontology Molecular Function:
aminomethyltransferase activity
Gene Ontology Cellular Component:
glycine cleavage complex
mitochondrial matrix
mitochondrion
Keywords:
Complete proteome
Lipoyl
Mitochondrion
Polymorphism
Reference proteome
Transit peptide

Publication

PubMed ID:
1671321 2025283 15616553 15489334 21269460 24275569 25944712