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货号： bs-5019R-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-5019R-Cy7
- 英文名称
- Anti-ACAT1/Cy7
- 中文名称
- Cy7标记的乙酰辅酶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
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 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
- 交叉反应
- Human, Mouse, Rat, Dog, Rabbit,
- 产品应用
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/ml
- 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.
- 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.
Similarity:
Belongs to the thiolase family.
Database links:Entrez Gene: 38Human
Entrez Gene: 110446Mouse
Entrez Gene: 25014Rat
Omim: 607809Human
SwissProt: P24752Human
SwissProt: Q8QZT1Mouse
SwissProt: P17764Rat
Unigene: 232375Human
Unigene: 293233Mouse
Unigene: 4054Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.