acetyl CoA acetyltransferase-1 (acetyl-CoA acetyltransferase mitochondrial, T2, ACAT1 ACAT MAT)

Function

plays a major role in ketone body metabolism

2 acetyl-CoA <--> CoA + acetoacetyl-CoA

Structure

• homotetramer -belongs to the thiolase family

Compartment

mitochondria

Pathology

• defects in ACAT1 are a cause of alpha-methylacetoaceticaciduria (beta-ketothiolase deficiency)

More general terms

• acetoacetyl CoA thiolase
• mitochondrial protein

Database

• OMIM: https://mirror.omim.org/entry/203750
• OMIM: https://mirror.omim.org/entry/607809
• UniProt: http://www.uniprot.org/uniprot/P24752.html
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=38