Mouse anti-Human ACAT1 Monoclonal Antibody | anti-ACAT1 antibody

Mouse Monoclonal [clone AT1.H11] (IgG) to Human ACAT1

Cat. Num. AAA246376

• Gene Names: ACAT1; T2; MAT; ACAT; THIL
• Reactivity: Human
• Applications: Immunohistochemistry, Western Blot, ELISA
• Purity: Immunoaffinity Purified
• Synonyms: ACAT1; Monoclonal Antibody; Mouse Monoclonal [clone AT1.H11] (IgG) to Human ACAT1; Anti-ACAT1 Antibody (N-Terminus; clone AT1.H11) IHC-plus; ACAT; Acetoacetyl-CoA thiolase; Acetyl-CoA acetyltransferase 1; MAT; T2; THIL; Human ACAT1; anti-ACAT1 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG
• Clone Number: AT1.H11
• Specificity: Recognize specifically human ACAT1. Other species not tested.
• Purity/Purification: Immunoaffinity Purified
• Form/Format: PBS, pH 7.4, 0.05% sodium azide. Sourced in Ascites.
• Concentration: 1 mg/ml (varies by lot)
• Sequence Length: 427

• Applicable Applications for anti-ACAT1 antibody: Immunohistochemistry (IHC - Paraffin), Western Blot (WB), ELISA (EIA)
• Application Notes: ELISA (1:2000), IHC-P (10 ug/ml), WB (1:1000)
• Target Species: Human
• Immunogen Description: Recombinant protein
• Immunogen Type: Recombinant protein
• Antigen Modification: N-Terminus
• Preparation and Storage: Short term 4 degree C, long term aliquot and store at -20 degree C, avoid freeze thaw cycles.

Immunohistochemistry (IHC)

(Anti-ACAT1 antibody IHC of human adrenal cortex. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 10 ug/ml.)

Related Product Information for anti-ACAT1 antibody

The ACAT1 mRNA encodes a mitochondrially localized enzyme that catalyzes the reversible formation of acetoacetyl-CoA from two molecules of acetyl-CoA. The ACAT gene spans approximately 27 kb and contains 12 exons interrupted by 11 introns. Defects in this gene 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 and Uniprot Product Information

• NCBI GI #: 4557237
• NCBI GeneID: 38
• NCBI Accession #: NP_000010.1
• NCBI GenBank Nucleotide #: NM_000019.3
• UniProt Accession #: P24752; Q96FG8; B2R6H1; G3XAB4
• Molecular Weight: 17,175 Da
• NCBI Official Full Name: acetyl-CoA acetyltransferase, mitochondrial
• NCBI Official Synonym Full Names: acetyl-CoA acetyltransferase 1
• NCBI Official Symbol: ACAT1
• NCBI Official Synonym Symbols: T2; MAT; ACAT; THIL
• NCBI Protein Information: acetyl-CoA acetyltransferase, mitochondrial; acetoacetyl-CoA thiolase; acetoacetyl Coenzyme A thiolase; acetyl-Coenzyme A acetyltransferase 1; mitochondrial acetoacetyl-CoA thiolase
• UniProt Protein Name: Acetyl-CoA acetyltransferase, mitochondrial
• UniProt Gene Name: ACAT1
• UniProt Synonym Gene Names: ACAT; MAT
• UniProt Entry Name: THIL_HUMAN

NCBI Description

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 Description

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: Lipid Metabolism - fatty acid; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Amino Acid Metabolism - tryptophan; Carbohydrate Metabolism - pyruvate; EC 2.3.1.9; Mitochondrial; Acetyltransferase; Lipid Metabolism - synthesis and degradation of ketone bodies; Carbohydrate Metabolism - butanoate; Secondary Metabolites Metabolism - terpenoid backbone biosynthesis; Amino Acid Metabolism - lysine degradation; Carbohydrate Metabolism - propanoate

Chromosomal Location of Human Ortholog: 11q22.3

Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial inner membrane

Molecular Function: protein homodimerization activity; enzyme binding; 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; cellular lipid metabolic process; brain development; branched chain family amino acid catabolic process; liver development; response to organic cyclic substance; protein homooligomerization

Disease: Alpha-methylacetoacetic Aciduria