This gene encodes a mitochondrially localized enzyme that catalyzes the reversible formation of acetoacetyl-CoA from two molecules of acetyl-CoA. Defects in this gene are associated with 3-ketothiolase deficiency, an inborn error of isoleucine catabolism characterized by urinary excretion of 2-methyl-3-hydroxybutyric acid, 2-methylacetoacetic acid, tiglylglycine, and butanone.
Categories
Primary Antibodies
Cellular Localization
Mitochondria
Clonality
polyclonal
Description
Plays a major role in ketone body metabolism.
Host
Rabbit
Immunogen
acetyl-Coenzyme A acetyltransferase 1
Isotype
IgG
Molecular Weight
40 kDa
Reactivity
Human, Mouse
Regulatory
RUO
Subcellular Location
mitochondrion
Synonyms
ACAT, ACAT1, Acetoacetyl CoA thiolase, MAT, T2, THIL
Uniprot
P24752
Gene Id
38
Research Area
Metabolism
Weight
17kDa,observed 45kDa
Form
liquid
Format
liquid
Purification
Immunogen affinity purified
Purity
>=95% as determined by SDS-PAGE
Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20°C for 12 months (Avoid repeated freeze / thaw cycles.)