Anti-FH/Fumarase

Fumarase (FH) is an enzyme that catalyzes the reversible hydration/dehydration of fumarate to malate in the mitochondria. Fumarase deficiency is caused by a mutation in the fumarase gene in humans. Fumarase deficiency is one of the few known deficiencies of the Krebs cycle or tricarboxylic acid cycle, the main enzymatic pathway of cellular aerobic respiration. Protein function: Catalyzes the reversible stereospecific interconversion of fumarate to L-malate (PubMed:30761759). Experiments in other species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other (Probable). [The UniProt Consortium] Recommended dilutions: WB 1:1,000.