catalog number :
MBS626693
products full name :
ACAT1, CT (Acetyl-CoA Acetyltransferase, Mitochondrial, Acetoacetyl-CoA Thiolase, T2, ACAT, MAT)
products short name :
ACAT1
products name syn :
Anti -ACAT1, CT (Acetyl-CoA Acetyltransferase, Mitochondrial, Acetoacetyl-CoA Thiolase, T2, ACAT, MAT)
other names :
acetyl-CoA acetyltransferase, mitochondrial; Acetyl-CoA acetyltransferase, mitochondrial; acetyl-CoA acetyltransferase, mitochondrial; acetoacetyl-CoA thiolase; acetoacetyl Coenzyme A thiolase; acetyl-Coenzyme A acetyltransferase 1; mitochondrial acetoacetyl-CoA thiolase; acetyl-CoA acetyltransferase 1; Acetoacetyl-CoA thiolase; T2
other gene names :
ACAT1; ACAT1; T2; MAT; ACAT; THIL; ACAT; MAT
uniprot entry name :
THIL_HUMAN
specificity :
Recognizes human ACAT1.
purity :
Purified. Purified by ammonium sulfate precipitation.
form :
Supplied as a liquid in PBS, 0.09% sodium azide.
storage stability :
May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
tested application :
ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC)
app notes :
Suitable for use in ELISA, Western Blot, and Immunohistochemistry. Dilution: ELISA: 1:1000. Western Blot: 1:50-1:100. Immunohistochemistry: 1:10-1:50
other info1 :
Immunogen: KLH-conjugated synthetic peptide corresponding to a portion of amino acids within residues 305-335 in the C-terminal region of human ACAT1.
products categories :
Antibodies; Abs to Enzymes, Acetyltransferase
products description :
ACAT1 is a mitochondrially localized enzyme that catalyzes the reversible formation of acetoacetyl-CoA from two molecules of acetyl-CoA. Defects in the gene encoding ACAT1 are associated with the alpha-methylacetoaceticaciduria disorder,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 mol weight :
45,200 Da
ncbi pathways :
Branched-chain Amino Acid Catabolism Pathway (106179); 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: Amino Acid Metabolism - tryptophan; Secondary Metabolites Metabolism - terpenoid backbone biosynthesis; Lipid Metabolism - fatty acid; Carbohydrate Metabolism - pyruvate; Carbohydrate Metabolism - butanoate; EC 2.3.1.9; Acetyltransferase; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Lipid Metabolism - synthesis and degradation of ketone bodies; Carbohydrate Metabolism - propanoate; Amino Acid Metabolism - lysine degradation. Chromosomal Location of Human Ortholog: 11q22.3. Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial inner membrane. Molecular Function: enzyme binding; protein homodimerization activity; acetyl-CoA C-acetyltransferase activity; metal ion binding; coenzyme binding. Biological Process: response to starvation; ketone body catabolic process; response to hormone stimulus; ketone body biosynthetic process; ketone body metabolic process; branched chain family amino acid catabolic process; brain development; cellular lipid metabolic process; liver development; response to organic cyclic substance; protein homooligomerization. Disease: Alpha-methylacetoacetic Aciduria