产品信息

蛋白质全称	3-羟基异丁酸脱氢酶
别名	HIBADH, mitochondrial, NS5ATP1, EC 1.1.1.31, MGC40361
Uniprot ID	Q99L13
免疫原	小鼠HIBADH重组蛋白
抗体亚型	IgG
预测分子量./观测分子量.	35 kDa / 30 kDa
纯化方式	亲和纯化

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应用

应用	物种	稀释	阳性样品
WB 免疫印迹	小鼠, 大鼠	1: 500-1: 1000	肝, 肾

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背景

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.

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图像

WB检测HIBADH蛋白(货号 GB111712). 样品: 经RIPA裂解液(货号G2002)处理的蛋白质. 封闭: 封闭: 3%脱脂牛奶(货号GC310001)溶于TBST溶液, 室温孵育1小时. —抗: 1: 500稀释, 4℃ 孵育过夜. 二抗: HRP标记山羊抗兔IgG (H+L) (货号GB23303), 1: 5000稀释, 室温孵育1小时.

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储存

储存条件	在-20℃下储存一年, 避免反复冻融.
储存缓冲液	含0.15% ProClin300防腐剂, 100 μg/mL牛血清白蛋白和50%甘油的磷酸盐缓冲液.

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注意:

1.本产品仅供研究使用.

2.建议用一抗稀释液(G2025)稀释本产品.