Molecular weight (DA)
45kD
Immunogen
The antiserum was produced against synthesized peptide derived from human ACAT1. AA range:221-270
Specificity
ACAT-1 Polyclonal Antibody detects endogenous levels of ACAT-1 protein.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/40000.. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
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. [provided by RefSeq, Feb 2009],
Function
catalytic activity:2 acetyl-CoA = CoA + acetoacetyl-CoA.,disease:Defects in ACAT1 are a cause of 3-ketothiolase deficiency (3KTD) [MIM:203750]; also known as alpha-methylacetoaceticaciduria. 3KTD is an 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.,enzyme regulation:Activated by potassium ions, but not sodium ions.,function:Plays a major role in ketone body metabolism.,similarity:Belongs to the thiolase family.,subunit:Homotetramer.,
Protein name
Acetyl-CoA acetyltransferase mitochondrial
Other name
ACAT1; ACAT; MAT; Acetyl-CoA acetyltransferase; mitochondrial; Acetoacetyl-CoA thiolase; T2
Fields
>>Fatty acid degradation;>>Valine, leucine and isoleucine degradation;>>Lysine degradation;>>Tryptophan metabolism;>>Pyruvate metabolism;>>Glyoxylate and dicarboxylate metabolism;>>Butanoate metabolism;>>Terpenoid backbone biosynthesis;>>Metabolic pathways;>>Carbon metabolism;>>Fatty acid metabolism;>>Fat digestion and absorption
Human protein sequence Database
P24752
Mouse protein sequence database
Q8QZT1
Rat protein sequence database
P17764
Cellular localization
Mitochondrion .
Tissue expression
Adipocyte,Brain,Fetal brain cortex,
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