HIBADH 抗体 (AA 34-332)
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Quick Overview for HIBADH 抗体 (AA 34-332) (ABIN7874076)
抗原
See all HIBADH 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 34-332
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原理
- HIBADH Antibody / 3-Hydroxyisobutyrate dehydrogenase
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纯化方法
- Immunogen affinity purified
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免疫原
- E.coli-derived human HIBADH recombinant protein (Position: R34-E332) was used as the immunogen for the HIBADH antibody.
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亚型
- IgG
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应用备注
- Optimal dilution of the HIBADH antibody should be determined by the researcher.
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限制
- 仅限研究用
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状态
- Lyophilized
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溶解方式
- Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL
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缓冲液
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.
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储存条件
- 4 °C,-20 °C
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储存方法
- After reconstitution, the HIBADH antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.
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- HIBADH (3-hydroxyisobutyrate Dehydrogenase (HIBADH))
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别名
- HIBADH
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背景
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HIBADH antibody detects 3-hydroxyisobutyrate dehydrogenase, a mitochondrial enzyme that catalyzes a critical step in valine catabolism. Encoded by the HIBADH gene on chromosome 7q31.1, this enzyme converts 3-hydroxyisobutyrate to methylmalonate semialdehyde using NAD+ as a cofactor. HIBADH plays an important role in branched-chain amino acid degradation and energy production by linking valine metabolism to the tricarboxylic acid (TCA) cycle. It is primarily expressed in liver, kidney, muscle, and other energy-demanding tissues.
HIBADH localizes to the mitochondrial matrix, where it functions within the valine degradation pathway that ultimately generates succinyl-CoA for entry into the TCA cycle. This enzymatic step contributes to both energy generation and metabolic flexibility, especially during fasting or muscle exertion. In addition to its metabolic role, HIBADH helps regulate mitochondrial redox balance through NAD+/NADH interconversion. Mutations or deficiencies in this enzyme may disrupt amino acid catabolism and contribute to metabolic disorders involving organic acid accumulation.
The HIBADH antibody is used in metabolism, mitochondrial, and enzymology research to detect mitochondrial dehydrogenases and characterize amino acid catabolic pathways. Western blot analysis identifies a 35 kilodalton band corresponding to HIBADH, while immunofluorescence reveals punctate mitochondrial staining. This antibody provides a valuable reagent for studying mitochondrial metabolism, oxidative phosphorylation, and amino acid utilization under physiological and disease conditions.
Beyond energy metabolism, altered HIBADH expression has been linked to mitochondrial dysfunction, muscle wasting, and cancer metabolism. Its regulation reflects cellular metabolic adaptation to nutrient and energy demands. The HIBADH antibody is validated by NSJ Bioreagents for western blotting and flow cytometry, providing consistent performance for the analysis of mitochondrial enzymatic function and metabolic flux regulation. -
UniProt
- P31937
抗原
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