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货号： bs-5070R-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-5070R-Cy7
- 英文名称
- Anti-HMGCS2/Cy7
- 中文名称
- Cy7标记的三羟基三甲基辅酶A合成酶2抗体
- 别 名
- 3 hydroxy 3 methylglutaryl Coenzyme A synthase 2 (mitochondrial); 3 hydroxy 3 methylglutaryl Coenzyme A synthase 2; 3 hydroxy 3 methylglutaryl coenzyme A synthase; 3-hydroxy-3-methylglutaryl coenzyme A synthase; HMCS2_HUMAN; HMG CoA synthase; HMG-CoA synthase; HMGCS 2; HMGCS2; Hydroxymethylglutaryl CoA synthase; Hydroxymethylglutaryl CoA synthase mitochondrial; Hydroxymethylglutaryl-CoA synthase; mitochondrial.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 心血管 细胞生物 免疫学 信号转导 脂蛋白 线粒体
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit,
- 产品应用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 52kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human HMGCS2
- 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:
HMGCS2 is a mitochondrial enzyme that catalyzes the first reaction of ketogenesis, a metabolic pathway that provides lipid-derived energy for various organs during times of carbohydrate deprivation, such as fasting. Mutations in this gene are associated with HMG-CoA synthase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.
Function:
This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase.
Subcellular Location:
Mitochondrion.
Tissue Specificity:
High expression in liver and colon. Low expression in testis, heart, skeletal muscle and kidney.
DISEASE:
Defects in HMGCS2 are the cause of HMG-CoA synthase deficiency (HMGCS deficiency) [MIM:605911]; also known as deficiency of mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase 2. Affected individuals present with severe hypoketotic hypoglycemia, mild hepatomegaly, or fatty liver, and a nondiagnostic pattern of urinary organic acids with increase of medium and short chain dicarboxylic acids.
Similarity:
Belongs to the HMG-CoA synthase family.
Database links:
UniProtKB/Swiss-Prot: P54868.1
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.