| 產(chǎn)品編號(hào) | bs-15483R |
| 英文名稱 | HIBADH Rabbit pAb |
| 中文名稱 | 3-羥基異丁酸脫氫酶抗體 |
| 別 名 | 3 hydroxy 2 methylpropanoate:NAD(+) oxidoreductase; 3 hydroxyisobutyrate dehydrogenase; 3 hydroxyisobutyrate dehydrogenase mitochondrial; EC 1.1.1.31; MGC40361; NS5ATP1; 3HIDH_HUMAN. |
| 研究領(lǐng)域 | 細(xì)胞生物 信號(hào)轉(zhuǎn)導(dǎo) 新陳代謝 線粒體 |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 克 隆 號(hào) | |
| 交叉反應(yīng) | Human,Mouse (predicted: Rat,Sheep,Cow,Horse) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 32 kDa |
| 檢測(cè)分子量 | |
| 細(xì)胞定位 | 細(xì)胞漿 線粒體 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human HIBADH: 251-336/336 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
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 |
| 產(chǎn)品圖片 |
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
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