ACAT1,乙酰辅酶A酰基转移酶1抗体

产品编号HZ-5019R
英文名称ACAT1
中文名称乙酰辅酶A酰基转移酶1抗体
别 名ACAT 1; ACAT; Acetoacetyl CoA thiolase; Acetyl CoA acetyltransferase, mitochondrial; Acetyl Coenzyme A acetyltransferase 1; MAT; RATACAL; T2; THIL; THIL_HUMAN; Acetyl-CoA acetyltransferase, mitochondrial; Acetoacetyl-CoA thiolase; T2.
规格价格0.1ml 0.2ml 
说 明 书0.1ml 0.2ml
研究领域肿瘤 细胞生物 免疫学 转录调节因子 线粒体
抗体来源Rabbit
克隆类型Polyclonal
ACAT1,乙酰辅酶A酰基转移酶1抗体交叉反应Human, Mouse, Rat, Dog, Rabbit,
产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量47kDa
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human ACAT1
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
ACAT1,乙酰辅酶A酰基转移酶1抗体PubMedPubMed
产品介绍background:
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].

Function:
Plays a major role in ketone body metabolism.

Subunit:
Homotetramer.

Subcellular Location:
Mitochondrion.

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.

ACAT1,乙酰辅酶A酰基转移酶1抗体Similarity:
Belongs to the thiolase family.

Gene ID:
38

Database links:
Entrez Gene: 38 Human
Entrez Gene: 110446 Mouse
Entrez Gene: 25014 Rat
Omim: 607809 Human
SwissProt: P24752 Human
SwissProt: Q8QZT1 Mouse
SwissProt: P17764 Rat
Unigene: 232375 Human
Unigene: 293233 Mouse
Unigene: 4054 Rat

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.