ACAT1
GeneName
ACAT1
Summary
ACAT1, also known as ACAT, MAT, or ACAT-1, is a 45kDa enzyme primarily located in the endoplasmic reticulum and mitochondrial matrix. It plays a pivotal role in lipid metabolism by catalysing the acylation of cholesterol and other sterols, facilitating the formation of cholesteryl esters. This enzyme is involved in several metabolic pathways, including the biosynthesis and catabolism of acetyl-CoA, as well as the metabolism of ketone bodies and fatty acids. ACAT1 is expressed in various tissues, including the liver and adipose tissue, and is crucial for maintaining cellular lipid homeostasis.
Importance
ACAT1 is relevant to: - Lipid metabolism and cholesterol homeostasis, impacting cardiovascular health and atherosclerosis - Adipose tissue development and energy storage, influencing obesity and metabolic disorders - Ketone body metabolism, which is significant in conditions such as diabetes and starvation - Hormonal responses and metabolic adaptations, affecting overall metabolic health and disease states
Top Products
For researchers investigating ACAT1, we highly recommend the top-selling recombinant monoclonal antibody, Anti-ACAT1 antibody [EPR10359] (ab168342). This antibody has been validated for use in Western blotting (WB), immunohistochemistry (IHC), and immunoprecipitation (IP), making it a versatile tool for various experimental needs. With 19 citations, it has garnered attention in the research community, reflecting its reliability and effectiveness in ACAT1 studies. This product is an excellent choice for those seeking consistent performance in their experiments. The Recombinant Human ACAT1 protein ELISA Kit (ab137175) is an excellent option for researchers looking to measure ACAT1 levels in their samples.
Abcam Product Citation Summary
The data indicates that ACAT1 is primarily studied in the context of cholesterol and fatty acid metabolism, with a focus on human cell lines and tissues, as well as mouse liver samples. The use of western blotting as the application suggests a strong interest in protein expression levels related to these metabolic processes.
Abcam Product Citation Table
Function
This is one of the enzymes that catalyzes the last step of the mitochondrial beta-oxidation pathway, an aerobic process breaking down fatty acids into acetyl-CoA (PubMed:1715688, PubMed:7728148, PubMed:9744475). Using free coenzyme A/CoA, catalyzes the thiolytic cleavage of medium- to long-chain 3-oxoacyl-CoAs into acetyl-CoA and a fatty acyl-CoA shortened by two carbon atoms (PubMed:1715688, PubMed:7728148, PubMed:9744475). The activity of the enzyme is reversible and it can also catalyze the condensation of two acetyl-CoA molecules into acetoacetyl-CoA (PubMed:17371050). Thereby, it plays a major role in ketone body metabolism (PubMed:1715688, PubMed:17371050, PubMed:7728148, PubMed:9744475).
Involvement in disease
3-ketothiolase deficiency
3KTD
An autosomal recessive inborn error of isoleucine catabolism characterized by intermittent ketoacidotic attacks associated with unconsciousness. Some patients die during an attack or are mentally retarded. Urinary excretion of 2-methyl-3-hydroxybutyric acid, 2-methylacetoacetic acid, triglylglycine, butanone is increased. It seems likely that the severity of this disease correlates better with the environmental or acquired factors than with the ACAT1 genotype.
None
The disease is caused by variants affecting the gene represented in this entry.
Pathway
Lipid metabolism; fatty acid beta-oxidation.
Post-translational modifications
Succinylation at Lys-268, adjacent to a coenzyme A binding site. Desuccinylated by SIRT5 (By similarity).
Sequence Similarities
Belongs to the thiolase-like superfamily. Thiolase family.
Cellular localization
- Mitochondrion
Alternative names
ACAT, MAT, ACAT1, Acetoacetyl-CoA thiolase, T2