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货号： bs-15483R-PE-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-15483R-PE-Cy7
- 英文名称
- Anti-HIBADH/PE-Cy7
- 中文名称
- PE-Cy7标记的3-羟基异丁酸脱氢酶抗体
- 别 名
- 3 hydroxy 2 methylpropanoate:NAD(+) oxidoreductase; 3 hydroxyisobutyrate dehydrogenase; 3 hydroxyisobutyrate dehydrogenase mitochondrial; EC 1.1.1.31; MGC40361; NS5ATP1; 3HIDH_HUMAN.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 信号转导 新陈代谢 线粒体
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Cow, Horse, Sheep,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 32kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human HIBADH
- 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:
HIBADH is a 336 amino acid mitochondrial enzyme that catalyzes the NAD+-dependent, reversible oxidization of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7.0 and 10.0, with optimal activity between 8.8 and 9.0. It was previously hypothesized that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterized by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.
Subcellular Location:
Mitochondrion.
Tissue Specificity:
Detected in skin fibroblasts.
Similarity:
Belongs to the 3-hydroxyisobutyrate dehydrogenase family.
Database links:Entrez Gene: 11112 Human
Entrez Gene: 58875 Mouse
Entrez Gene: 63938 Rat
Omim: 608475 Human
SwissProt: P31937 Human
SwissProt: Q99L13 Mouse
SwissProt: P29266 Rat
Unigene: 406758 Human
Unigene: 286458 Mouse
Unigene: 73 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.