protein

ACAT1 Immunizing Peptide

MBS425066

0.1 mg

190 USD

Peptide

ACAT1 Immunizing Peptide

ACAT1

ACAT1; acetyl-Coenzyme A acetyltransferase 1; ACAT; MAT; T2; THIL; acetoacetyl Coenzyme A thiolase; ACAT1 (aa253-266)

acetyl-CoA acetyltransferase, mitochondrial; Acetyl-CoA acetyltransferase, mitochondrial; acetyl-CoA acetyltransferase, mitochondrial; acetyl-CoA acetyltransferase 1; Acetoacetyl-CoA thiolase; T2

ACAT1

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

THIL_HUMAN

Human, Mouse, Rat, Dog, Cow

427

C-DEEYKRVDFSKVPK

100ug of dried peptide

Shipped at ambient temperature, store at -20 degree C

4557237

NP_000010.1

NM_000019.3

17,175 Da

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)

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

0.1 mg

190 USD