2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial; AKB ligase; aminoacetone synthase; glycine acetyltransferase (GCAT, KBL)

Function

• amino-acid degradation
  • L-threonine degradation via oxydo-reductase pathway
  • glycine from L-threonine: step 2/2

Acetyl-CoA + glycine <--> CoA + 2-amino-3-oxobutanoate

Cofactor: pyridoxal phosphate (putative)

Structure

• belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family

Compartment

• mitochondria (putative), nucleus
• translocates to the nucleus upon cold & osmotic stress

Expression

expressed in heart, brain, liver, pancreas > lung

More general terms

• ligase or synthetase
• mitochondrial protein
• aminotransferase

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=23464
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:23464
• OMIM: https://mirror.omim.org/entry/607422
• UniProt: http://www.uniprot.org/uniprot/O75600.html