产品名称
ACAT-1 Rabbit Polyclonal Antibody
别名
ACAT1; ACAT; MAT; Acetyl-CoA acetyltransferase; mitochondrial; Acetoacetyl-CoA thiolase; T2
蛋白名称
Acetyl-CoA acetyltransferase mitochondrial
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=38
Human Swissprot No.
P24752
Human Swissprot Link
http://www.uniprot.org/uniprotkb/P24752/entry
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=110446
Mouse Swissprot No.
Q8QZT1
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q8QZT1
Rat Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=25014
Rat Swissprot Link
http://www.uniprot.org/uniprot/P17764
免疫原
The antiserum was produced against synthesized peptide derived from human ACAT1. AA range:221-270
特异性
ACAT-1 Polyclonal Antibody detects endogenous levels of ACAT-1 protein.
稀释度
WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:40000.. IF 1:50-200
宿主
Polyclonal, Rabbit,IgG
背景介绍
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],
组织表达
Adipocyte,Brain,Fetal brain cortex,
信号通路
Fatty acid metabolism;Synthesis and degradation of ketone bodies;Valine; leucine and isoleucine degradation;Lysine degradation;Tryptophan metabolism;Pyruvate metabolism;Propanoate metabolism;Butanoate metabolism;Terpenoid backbone biosynthesis;
功能
catalytic activity:2 acetyl-CoA = CoA + acetoacetyl-CoA.,disease:Defects in ACAT1 are a cause of 3-ketothiolase deficiency (3KTD) [MIM:203750]; 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.,enzyme regulation:Activated by potassium ions, but not sodium ions.,function:Plays a major role in ketone body metabolism.,similarity:Belongs to the thiolase family.,subunit:Homotetramer.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.