Mouse Monoclonal [clone AT1.H11] (IgG) to Human ACAT1 | MyBioSource MBS246376 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

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

catalog :

MBS246376

quantity :

0.05 mg

price :

495 USD

clonality :

monoclonal

host :

mouse

conjugate :

nonconjugated

clone name :

AT1.H11

reactivity :

human

application :

western blot, ELISA, immunohistochemistry, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS246376

products type :

Antibody

products full name :

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

products short name :

ACAT1

products name syn :

Anti-ACAT1 Antibody (N-Terminus, clone AT1.H11) IHC-plus; ACAT1; ACAT; Acetoacetyl-CoA thiolase; Acetyl-CoA acetyltransferase 1; MAT; T2; THIL; Human ACAT1

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

products gene name :

ACAT1

other gene names :

ACAT1; ACAT1; T2; MAT; ACAT; THIL; ACAT; MAT

uniprot entry name :

THIL_HUMAN

clonality :

Monoclonal

isotype :

IgG

clone :

AT1.H11

host :

Mouse

reactivity :

Human

sequence length :

427

specificity :

Recognize specifically human ACAT1. Other species not tested.

purity :

Immunoaffinity Purified

form :

PBS, pH 7.4, 0.05% sodium azide. Sourced in Ascites.

concentration :

1 mg/ml

storage stability :

Short term 4 degree C, long term aliquot and store at -20 degree C, avoid freeze thaw cycles.

tested application :

Immunohistochemistry (IHC - Paraffin), Western Blot (WB), ELISA (EIA)

app notes :

ELISA (1:2000), IHC-P (10 ug/ml), WB (1:1000)

other info1 :

Target Species: Human. Immunogen Description: Recombinant protein

other info2 :

Immunogen Type: Recombinant protein. Antigen Modification: N-Terminus

products description :

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 gi num :

4557237

ncbi acc num :

NP_000010.1

ncbi gb acc num :

NM_000019.3

uniprot acc num :

P24752

ncbi mol weight :

17,175 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: 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

size1 :

0.05 mg

price1 :

495 USD

more info or order :

MyBioSource product webpage