Anti-HIBADH antibody [EPR12519(B)] - BSA and Azide free

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

HIBADH known as 3-hydroxyisobutyrate dehydrogenase plays a role in the catabolic pathway of valine. It catalyzes the oxidation of 3-hydroxyisobutyrate to 3-oxopropanoate. This enzyme weighs approximately 33 kDa. Expression of HIBADH occurs predominantly in the liver kidney and to a lesser extent the heart and skeletal muscle tissues. High expression in these organs suggests its importance in energy metabolism and intermediate metabolite processing.

Biological function summary

This enzyme facilitates the metabolism of amino acids by converting valine-derived intermediates. HIBADH contributes significantly to energy production particularly during periods of fasting. It is not typically recognized as part of a larger protein complex functioning independently within metabolic pathways. Its role in energy homeostasis indicates its essential part in maintaining cellular function during metabolic stress.

Pathways

HIBADH is integral to the valine degradation pathway and also impacts the broader branched-chain amino acid catabolism process. These pathways are critical for recycling amino acids for ATP production. HIBADH interacts with other enzymes like acyl-CoA dehydrogenases which further process breakdown products. This interaction highlights HIBADH's connection to a consistent and efficient metabolic network ensuring effective energy yield from valine.

Associated diseases and disorders

HIBADH has relevance to maple syrup urine disease and its metabolic management. Proper function of HIBADH is essential to avoid accumulation of toxic valine metabolites linked to this condition. Additionally connection to the protein BCKDHA hints at broader implications for amino acid metabolism disorders. BCKDHA part of the branched-chain alpha-ketoacid dehydrogenase complex indicates HIBADH's involvement in a critical intersection of metabolic control and disease prevention.