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Rabbit Anti-ACAT1/BF594 Conjugated antibody (bs-5019R-BF594)
訂購熱線:400-901-9800
訂購郵箱:sales@www.chomd.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@www.chomd.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-5019R-BF594
英文名稱 Rabbit Anti-ACAT1/BF594 Conjugated antibody
中文名稱 BF594標記的乙酰輔酶A酰基轉移酶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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  免疫學  轉錄調節因子  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Rabbit, )
產品應用 IF=1:50-200 
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/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ACAT1
亞    型 IgG
純化方法 affinity purified by Protein A
儲 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.
產品介紹 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-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.

Similarity:
Belongs to the thiolase family.

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.
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