基因名:
HIBADH
产品别名:
NS5ATP1; HIBADH; 3-hydroxyisobutyrate dehydrogenase; 3-hydroxyisobutyrate dehydrogenase; 3-hydroxyisobutyrate dehydrogenase, mitochondrial; 3'-hydroxyisobutyrate dehydrogenase; epididymis secretory sperm binding protein; 3-羟基异丁酸脱氢酶; 3-羟基异丁酸脱氢酶(HIBADH);
背景信息:
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.
标签:His-tag
分类:Recombinant
类型:Protein
偶联物:Unconjugated
内毒素水平:按批次,参阅瓶身标签
性状:Liquid
浓度:Batch dependent (Please refer to the vial label for the specific concentration.)
纯化类型:purified
内含物:不含防腐剂
应用:Positive Control;Immunogen;SDS-PAGE;WB.
储存:2-8°C不超过一个月,-80°C不超过12个月。避免反复冻融。
注意事项:仅供实验室使用。不适用于人类或动物的任何临床,治疗或诊断用途。不适合动物或人类食用。