3-羟基异丁酸脱氢酶抗体
规格：1mg/1ml
英文名： HIBADH
别名： 3 hydroxy 2 methylpropanoate:NAD(+) oxidoreductase; 3 hydroxyisobutyrate dehydrogenase; 3 hydroxyisobutyrate dehydrogenase mitochondrial; EC 1.1.1.31; MGC40361; NS5ATP1; 3HIDH_HUMAN.
分子量： 32kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human HIBADH
交叉反应：Human, Mouse, Rat, Cow, Horse, Sheep,
细胞定位：
3-羟基异丁酸脱氢酶抗体产品介绍：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 3-羟基异丁酸脱氢酶抗体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 Entrez3-羟基异丁酸脱氢酶抗体 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.
产3-羟基异丁酸脱氢酶抗体品应用：WB=1:100-500 ELISA=1:500-1000 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.
研究领域：细胞生物 信号转导 新陈代谢 线粒体
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid