产品编号 | bs-15483R |
英文名称 | HIBADH Rabbit pAb |
中文名称 | 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. |
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, (predicted: Rat, Cow, Horse, Sheep, ) |
产品应用 | WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复)
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
理论分子量 | 32 kDa |
检测分子量 | |
细胞定位 | 细胞浆 线粒体 |
性 状 | Liquid |
浓 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human HIBADH: 251-336/336 |
亚 型 | IgG |
纯化方法 | affinity purified by Protein A |
缓 冲 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
保存条件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
PubMed | PubMed |
产品介绍 |
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. SWISS: P31937 Gene ID: 11112 Database links: Entrez Gene: 11112 Human Entrez Gene: 58875 Mouse Omim: 608475 Human SwissProt: P31937 Human SwissProt: Q99L13 Mouse Unigene: 406758 Human Unigene: 286458 Mouse Unigene: 73 Rat |
产品图片 |
Sample:
193T(Human) Cell Lysate at 30 ug
MCF-7(Human) Cell Lysate at 30 ug
HepG2(Human) Cell Lysate at 30 ug
Kidney (Mouse) Lysate at 40 ug
Primary: Anti- HIBADH (bs-15483R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 32 kD
Observed band size: 30 kD
|
1、抗体溶解方法 | |
2、抗体修复方式 | |
3、常用试剂的配制 | |
4、免疫组化操作步骤 | |
5、免疫组化问题解答 | |
6、Western Blotting 操作步骤 | |
7、Western Blotting 问题解答 | |
8、关于肽链的设计 | |
9、多肽的溶解与保存 | |
10、酶标抗体效价测定程序 | |