OnCo

FH (Fumarate hydratase, mitochondrial) is an enzyme. The public catalogues list it as a drug target, a biomarker and a DNA repair gene, and clinical evidence ties its variants to diagnosis, prognosis or drug response. Tied to Neuroendocrine tumours, Skin cancer, Papillary renal cell carcinoma and 1 more. This dossier gathers the 0 products (0 approved), 0 trials, 0 pathways and 0 resistance routes in the corpus that involve it, with external identifiers so it can be joined to UniProt, ChEMBL, Open Targets and the rest of biology.

Biology

Catalyses the reversible stereospecific interconversion of fumarate to L-malate. Experiments in other species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other. Catalyses the hydration of fumarate to L-malate in the tricarboxylic acid (TCA) cycle to facilitate a transition step in the production of energy in the form of NADH. Catalyses the dehydration of L-malate to fumarate. Fumarate metabolism in the cytosol plays a role during urea cycle and arginine metabolism; fumarate being a by-product of the urea cycle and amino-acid catabolism. Also plays a role in DNA repair by promoting non-homologous end-joining (NHEJ). Location: Mitochondrion; Cytoplasm, cytosol; Nucleus; Chromosome (UniProt). Locus 1q43 (HGNC).

Where it is found
  • Neuroendocrine tumours: Open Targets association 0.54 with neuroendocrine neoplasm (MONDO_0019496)
  • Skin cancer: Open Targets association 0.51 with skin cancer (MONDO_0002898)
  • Papillary renal cell carcinoma: CIViC evidence names this disease
  • Melanoma: Open Targets association 0.51 with melanoma (MONDO_0005105)
Class: enzyme · Gene: FH · Facts checked 2026-09-23 · Target page

External identifiers

Built from HGNC, Ensembl, UniProt and ChEMBL ids

Products by modality and phase

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Resistance routes

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Companion diagnostics

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Literature

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Query for this target: (TITLE:"FH" OR ABSTRACT:"FH" OR TITLE:"fumarate hydratase" OR ABSTRACT:"fumarate hydratase" OR TITLE:"Fumarate hydratase, mitochondrial" OR ABSTRACT:"Fumarate hydratase, mitochondrial") AND (cancer OR tumor OR tumour OR oncology OR carcinoma OR lymphoma OR leukemia OR leukaemia OR myeloma OR sarcoma OR melanoma OR glioma). Results are unfiltered search hits about FH, not a curated reading list.

Export

The dossier as machine-readable JSON: identifiers from HGNC, Ensembl, UniProt and ChEMBL, products with status, trials, pathways, hotspots, open questions and assays. The full entity record is in the open API at /api/v1/entities/fh.json. Licence CC BY-NC 4.0.