Fumarate hydratase (FH), encoded by the FH gene, is an enzyme which catalyses conversion of fumarate to L-malate as part tricarboxylic acid cycle. Biallelic germline mutations in result fumaric aciduria, a metabolic disorder resulting severe neurological and developmental abnormalities. Heterozygous hereditary leiomyomatosis renal cell carcinoma, cancer predisposition syndrome. deficiency has m...