產(chǎn)品編號(hào) yb-5019R
英文名稱 Anti-ACAT1抗體
中文名稱 *?；D(zhuǎn)移酶1抗體
別    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.
Anti-ACAT1抗體 
說 明 書 0.2ml  
研究領(lǐng)域 腫瘤  細(xì)胞生物  免疫學(xué)  轉(zhuǎn)錄調(diào)節(jié)因子  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human, Mouse, Rat, Dog, Rabbit, 
產(chǎn)品應(yīng)用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復(fù)） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 47kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human ACAT1
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.

PubMed PubMed
產(chǎn)品介紹 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-methylacetouria. 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.


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