Acetyl Coenzyme A Acetyltransferase 1, ELISA Kit

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

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Small volumes of ACAT1 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS098024 is a ready-to-use microwell, strip-or-full plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Acetyl Coenzyme A Acetyltransferase 1 (ACAT1) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing ACAT1. The ELISA analytical biochemical technique of the MBS098024 kit is based on ACAT1 antibody-ACAT1 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect ACAT1 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, ACAT1. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

45,200 Da

acetyl-CoA acetyltransferase 1

acetyl-CoA acetyltransferase, mitochondrial; acetoacetyl-CoA thiolase; acetoacetyl Coenzyme A thiolase; acetyl-Coenzyme A acetyltransferase 1; mitochondrial acetoacetyl-CoA thiolase

Acetyl-CoA acetyltransferase, mitochondrial

ACAT; MAT  [Similar Products]

THIL_HUMAN

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]

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

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; brain development; ketone body metabolic process; cellular lipid metabolic process; branched chain family amino acid catabolic process; liver development; response to organic cyclic substance; protein homooligomerization

Disease: Alpha-methylacetoacetic Aciduria

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