Names & Taxonomy

Uniprot ID:
O75600
Entry Name:
KBL_HUMAN
Status:
reviewed
Protein Names:
2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial (AKB ligase) (EC 2.3.1.29) (Aminoacetone synthase) (Glycine acetyltransferase)
Gene Names:
GCAT KBL
Gene Names Primary:
GCAT
Organism:
Homo sapiens (Human)

Structure

Length:
419
Sequence:
MWPGNAWRAALFWVPRGRRAQSALAQLRGILEGELEGIRGAGTWKSERVITSRQGPHIRVDGVSGGILNFCANNYLGLSSHPEVIQAGLQALEEFGAGLSSVRFICGTQSIHKNLEAKIARFHQREDAILYPSCYDANAGLFEALLTPEDAVLSDELNHASIIDGIRLCKAHKYRYRHLDMADLEAKLQEAQKHRLRLVATDGAFSMDGDIAPLQEICCLASRYGALVFMDECHATGFLGPTGRGTDELLGVMDQVTIINSTLGKALGGASGGYTTGPGPLVSLLRQRARPYLFSNSLPPAVVGCASKALDLLMGSNTIVQSMAAKTQRFRSKMEAAGFTISGASHPICPVMLGDARLASRMADDMLKRGIFVIGFSYPVVPKGKARIRVQISAVHSEEDIDRCVEAFVEVGRLHGALP
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Mitochondrion

Function

Pathway:
Amino-acid degradation; L-threonine degradation via oxydo-reductase pathway; glycine from L-threonine: step 2/2.
Catalytic Activity:
Acetyl-CoA + glycine = CoA + 2-amino-3-oxobutanoate.
Cofactor:
COFACTOR: Name=pyridoxal 5'-phosphate; Xref=ChEBI:CHEBI:597326;
Cross Reference Drug Bank:
DB00145
Gene Ontology Go:
mitochondrial inner membrane
mitochondrion
nucleoplasm
glycine C-acetyltransferase activity
pyridoxal phosphate binding
biosynthetic process
cellular amino acid metabolic process
L-threonine catabolic process to glycine
Gene Ontology Biological Process:
biosynthetic process
cellular amino acid metabolic process
L-threonine catabolic process to glycine
Gene Ontology Molecular Function:
glycine C-acetyltransferase activity
pyridoxal phosphate binding
Gene Ontology Cellular Component:
mitochondrial inner membrane
mitochondrion
nucleoplasm
Keywords:
Acetylation
Acyltransferase
Alternative splicing
Complete proteome
Mitochondrion
Nucleus
Polymorphism
Pyridoxal phosphate
Reference proteome
Transferase
Transit peptide

Publication

PubMed ID:
10712613 14702039 10591208 15489334 17688197 21269460 24275569 25944712