Anti-HIBCH antibody

Supplementary info

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

Activity summary

HIBCH or 3-hydroxyisobutyryl-CoA hydrolase is an enzyme involved in the catabolic pathway of valine metabolism. It exhibits a molecular mass of approximately 37 kDa. This enzyme processes 3-hydroxyisobutyryl-CoA into free 3-hydroxyisobutyrate and CoA. HIBCH is expressed in several tissues with high levels found in liver and kidney reflecting its active role in amino acid metabolism.

Biological function summary

The enzyme catalyzes a specific reaction in the branched-chain amino acid metabolic pathway which is an essential process for the degradation and utilization of valine. HIBCH functions independently not forming part of any larger complex. This enzyme is key for maintaining the balance of metabolites derived from valine and is involved in energy production from protein breakdown.

Pathways

HIBCH plays a significant role in the valine degradation pathway where it acts downstream of other enzymes like acyl-CoA dehydrogenase. During this pathway it collaborates with various enzymes to ensure efficient energy extraction from valine. This process links valine metabolism to the larger citric acid cycle where it coordinates with enzymes such as isovaleryl-CoA dehydrogenase.

Associated diseases and disorders

Mutations or deficiencies in HIBCH correlate with 3-hydroxyisobutyryl-CoA hydrolase deficiency a metabolic disorder that can manifest as developmental delay neurological deficits or acute metabolic crises in severe cases. The enzyme has links to valine metabolism-related disorders where disturbances in its function can lead to elevated levels of neurotoxic intermediates. Its interaction with related enzymes such as branched-chain alpha-keto acid dehydrogenase further accentuates its importance in maintaining metabolic equilibrium.