# FH

Source: https://onco.cc/targets/fh/  
OnCo record `fh` (Target). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## Summary

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.

CIViC holds 2 clinical evidence items and 0 assertions across 2 variants, naming Erlotinib and Bevacizumab. Open Targets scores its association with cancer at 0.76 (direct and indirect evidence; datatypes literature 0.99, genetic association 0.67, somatic mutation 0.95, genetic literature 0.85).

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: fumarate hydratase; Fumarate hydratase, mitochondrial
- Tags: cancer-genes-wave
- Symbol: FH
- Class: enzyme
- 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 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)

## Notes

- Written by scripts/fetch-cancer-genes.ts from CIViC, Open Targets, IntOGen, HGNC and UniProt; the function text is UniProt's, condensed and in UK spelling. Roles: CIViC lists 2 therapies; CIViC holds 2 clinical evidence items on its variants; UniProt keyword "DNA repair". Evidence tier "clinical-evidence" is the strongest of those signals.
- Prevalence not recorded: none of the sources gives a positivity rate.
- Diseases the sources name that have no OnCo cancer page yet, so they are not linked: Hereditary Renal Cell Carcinoma.

## Sources

- HGNC HGNC:3700: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:3700
- UniProt P07954: https://www.uniprot.org/uniprotkb/P07954/entry
- NCBI Gene 2271: https://www.ncbi.nlm.nih.gov/gene/2271
- Ensembl ENSG00000091483: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000091483

## Connected records

- collections: [CIViC](https://onco.cc/collections/civic/), [Open Targets Platform](https://onco.cc/collections/open-targets/)
- cancers: [Melanoma](https://onco.cc/cancers/melanoma/), [Neuroendocrine tumours](https://onco.cc/cancers/neuroendocrine/), [Papillary renal cell carcinoma](https://onco.cc/cancers/papillary-rcc/), [Skin cancer (all types)](https://onco.cc/cancers/skin-cancer/)

---
JSON: https://onco.cc/api/v1/entities/fh.json