ACAT2 Protein Overview: Sequence, Structure, Function and Protein Interaction

ACAT2 Protein Overview

ACAT2 reagents

De Groot et al. (1977) reported a child, born of unrelated parents, who had normal development until about 3 to 4 months of age, when she showed a slowing in motor development and even some loss in acquired motor abilities. At age 14 months, she had poor head control and was unable to sit or roll over. She had ataxic and choreic movements, with involuntary vertical ocular movements. She also had severely impaired cognitive development at age 16 months. Laboratory studies showed increased serum lactate and pyruvate, and high levels of ketones on a ketogenic diet. This suggested impaired utilization of ketones. Liver biopsy showed low levels of cytosolic acetoacetyl-CoA thiolase (ACAT2; 100678), and the enzyme showed increased sensitivity to inhibition by CoA with decreased affinity for acetoacetyl-CoA compared to control. Liver biopsy also showed increased deposition of fat lipid droplets and glycogen accumulation. De Groot et al. (1977) postulated that the severe neurologic phenotype in this patient resulted from a disruption in lipid processing in the brain. Bennett et al. (1984) reported a boy, born of unrelated parents, who presented at age 7 months with hypotonia, pyrexia, and screaming, and was found to have severe developmental delay with abnormal EEG. Laboratory studies showed increased urinary ketones. Cultured fibroblasts showed a 50% reduction in activity of cytosolic acetoacetyl-CoA thiolase. A low-fat diet was instituted, which resulted in reduction of ketosis. After the low-fat diet was instituted, he developed severe gastrointestinal problems consistent with colitis cystica superficialis, which responded to steroid treatment.

ACAT2 protein family

Belongs to the thiolase family.

ACAT2 protein name

Recommended name
Acetyl-CoA acetyltransferase, cytosolic
acetoacetyl Coenzyme A thiolase
Alternative name
Acetyl-CoA transferase-like protein Cytosolic acetoacetyl-CoA thiolase

ACAT2 Protein Sequence

Species Human ACAT2 protein
Length 397
Mass (Da) 41351
Sequence Human ACAT2 protein sequence
Species Mouse ACAT2 protein
Length 397
Mass (Da) 41298
Sequence Mouse ACAT2 protein sequence
Species Rat ACAT2 protein
Length 397
Mass (Da) 41108
Sequence Rat ACAT2 protein sequence

ACAT2 Protein Molecular Weight & PI

Acetyl-CoA acetyltransferase, cytosolic (EC (Acetyl-CoA transferase-like protein) (Cytosolic acetoacetyl-CoA thiolase)Homo sapiens (Human).

The parameters have been computed for the following feature

FT CHAIN 1-397 Acetyl-CoA acetyltransferase, cytosolic.

Molecular weight (Da)


Theoretical pI


ACAT2 Protein Structure

Human cytosolic acetoacetyl-CoA thiolase complexed with CoA
2004-06-20   Released:  2005-03-01
Deposition Author(s)
Kursula, P., Fukao, T., Kondo, N., Wierenga, R.K.
Homo sapiens
Expression System
Escherichia coli
Experimental Data Snapshot
1.5500 Å
R-Value Free
R-Value Work
1WL4 From PDB

Human ACAT2 protein Secondary structure

ACAT2 Protein Interaction

Recombinant ACAT2 Protein Feature

ACAT2 Protein, Rat, Recombinant (His Tag)

High Purity
> 95 % as determined by SDS-PAGE.
Low Endotoxin
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