Anti-HIBADH / 3-Hydroxyisobutyrate dehydrogenase

Adding 0.2 ml of distilled water will yield a concentration of 500 ug/ml. 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.