FITC标记的3-羟基异丁酸脱氢酶抗体
产品名称： FITC标记的3-羟基异丁酸脱氢酶抗体
英文名称： Anti-HIBADH/FITC
产品编号： HZ-15483R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-HIBADH/FITC Conjugated antibody
FITC标记的3-羟基异丁酸脱氢酶抗体
| 英文名称 | Anti-HIBADH/FITC |
| 中文名称 | FITC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 |
HiBADH是一种336氨基酸的线粒体酶，催化3-羟基异丁酸酯与甲基丙二酸半醛的NAD+依赖性可逆氧化，缬氨酸分解代谢的中间产物。该酶在pH为7和10之间作为同源二聚体，在8.8和9之间具有最佳活性。先前推测，编码HiBADH的基因缺陷可能是3-羟基异丁酸尿症的病因，这是一种罕见的疾病，其特征是多种临床表现，如神经发育问题和畸形特征。然而，结果表明，与对照组相比，3-羟基异丁酸尿患者的HiBADH活性是相等的。