catalog number :
MBS766304
products full name :
ACAT1 Rabbit Polyclonal
products short name :
[ACAT1]
products name syn :
[ACAT, ACAT1, Acetoacetyl CoA thiolase, MAT, T2, THIL]
other names :
[acetyl-CoA acetyltransferase, mitochondrial; Acetyl-CoA acetyltransferase, mitochondrial; acetyl-CoA acetyltransferase, mitochondrial; acetyl-CoA acetyltransferase 1; Acetoacetyl-CoA thiolase; T2]
products gene name :
[ACAT1]
other gene names :
[ACAT1; ACAT1; T2; MAT; ACAT; THIL; ACAT; MAT]
uniprot entry name :
THIL_HUMAN
reactivity :
Human, Mouse; other species are not tested
specificity :
Please decide the specificity by homology
purity :
>=95% as determined by SDS-PAGE. Immunogen Affinity Purified
storage stability :
Store at -20° for 24 months (Avoid repeated freeze / thaw cycles.
tested application :
ELISA, WB, IHC
app notes :
WB: 1:500 - 1:2000. IHC: 1:50 - 1:200
image1 heading :
Western Blot (WB)
image2 heading :
Immunohistochemistry (IHC)
other info1 :
Immunogen: Acetyl-Coenzyme A acetyltransferase 1. Buffer: PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
other info2 :
Calculated MW: 40 kDa
products description :
This gene encodes a mitochondrially localized enzyme that catalyzes the reversible formation of cetoacetyl-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.
ncbi acc num :
NP_000010.1
ncbi gb acc num :
NM_000019.3
ncbi pathways :
Branched-chain Amino Acid Catabolism Pathway (1270161); Butanoate Metabolism Pathway (83007); Butanoate Metabolism Pathway (391); C5 Isoprenoid Biosynthesis, Mevalonate Pathway (413387); C5 Isoprenoid Biosynthesis, Mevalonate Pathway (468288); Carbon Metabolism Pathway (814926); Carbon Metabolism Pathway (817567); Fatty Acid Beta Oxidation Pathway (198865); Fatty Acid Degradation Pathway (82935); Fatty Acid Degradation Pathway (296)
ncbi summary :
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]
uniprot summary :
ACAT1: Plays a major role in ketone body metabolism. Defects in ACAT1 are a cause of 3-ketothiolase deficiency (3KTD); 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. Belongs to the thiolase family. Protein type: Carbohydrate Metabolism - pyruvate; Amino Acid Metabolism - tryptophan; EC 2.3.1.9; Secondary Metabolites Metabolism - terpenoid backbone biosynthesis; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Lipid Metabolism - fatty acid; Acetyltransferase; Carbohydrate Metabolism - butanoate; Carbohydrate Metabolism - propanoate; Mitochondrial; Amino Acid Metabolism - lysine degradation; Lipid Metabolism - synthesis and degradation of ketone bodies. Chromosomal Location of Human Ortholog: 11q22.3. Cellular Component: mitochondrial inner membrane; mitochondrial matrix; mitochondrion. Molecular Function: acetyl-CoA C-acetyltransferase activity; coenzyme binding; enzyme binding; metal ion binding; protein homodimerization activity. Biological Process: brain development; branched chain family amino acid catabolic process; ketone body biosynthetic process; ketone body catabolic process; liver development; protein homooligomerization; response to hormone stimulus; response to organic cyclic substance; response to starvation. Disease: Alpha-methylacetoacetic Aciduria