產(chǎn)品編號 | bs-15483R-Cy5.5 |
英文名稱 | Rabbit Anti-HIBADH/Cy5.5 Conjugated antibody |
中文名稱 | Cy5.5標記的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. |
規(guī)格價格 | 100ul/2980元 購買 大包裝/詢價 |
說 明 書 | 100ul |
研究領(lǐng)域 | 細胞生物 信號轉(zhuǎn)導(dǎo) 新陳代謝 線粒體 |
抗體來源 | Rabbit |
克隆類型 | Polyclonal |
交叉反應(yīng) | (predicted: Human, Mouse, Rat, Cow, Horse, Sheep, ) |
產(chǎn)品應(yīng)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 狀 | Lyophilized or Liquid |
濃 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human HIBADH |
亞 型 | IgG |
純化方法 | affinity purified by Protein A |
儲 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
保存條件 | 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. |
產(chǎn)品介紹 |
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 Omim: 608475 Human SwissProt: P31937 Human SwissProt: Q99L13 Mouse 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. |
1、抗體溶解方法 | |
2、抗體修復(fù)方式 | |
3、常用試劑的配制 | |
4、免疫組化操作步驟 | |
5、免疫組化問題解答 | |
6、Western Blotting 操作步驟 | |
7、Western Blotting 問題解答 | |
8、關(guān)于肽鏈的設(shè)計 | |
9、多肽的溶解與保存 | |
10、酶標抗體效價測定程序 | |
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