Recombinant Human 2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial (GCAT)

Uniprot Id

O75600

Target Species

Human

Target Name

GCAT

Target Full Name

2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial

Target Subcellular Location

Mitochondrion. Nucleus. Note=Translocates to the nucleus upon cold and osmotic stress.

Target Protein Families

Class-II pyridoxal-phosphate-dependent aminotransferase family

Target Tissue Specificity

Strongly expressed in heart, brain, liver and pancreas. Also found in lung.

Target Synonyms

2 amino 3 ketobutyrate CoA ligase ; 2 amino 3 ketobutyrate coenzyme A ligase, mitochondrial; 2-amino-3-ketobutyrate coenzyme A ligase; AKB ligase; Aminoacetone synthase; GCAT; Glycine acetyltransferase; glycine C acetyltransferase ; glycine C acetyltransferase (2 amino 3 ketobutyrate coenzyme A ligase); KBL_HUMAN; KLB; mitochondrial

Target Background

The degradation of L-threonine to glycine consists of a two-step biochemical pathway involving the enzymes L-threonine dehydrogenase and 2-amino-3-ketobutyrate coenzyme A ligase. L-Threonine is first converted into 2-amino-3-ketobutyrate by L-threonine dehydrogenase. This gene encodes the second enzyme in this pathway, which then catalyzes the reaction between 2-amino-3-ketobutyrate and coenzyme A to form glycine and acetyl-CoA. The encoded enzyme is considered a class II pyridoxal-phosphate-dependent aminotransferase. Alternate splicing results in multiple transcript variants. A pseudogene of this gene is found on chromosome 14.