fumarate hydratase
Sign in to saveAlso known as FH, epididymis secretory sperm binding protein, fumarate hydratase, mitochondrial, EF-3, fumarase
Fumarase (or fumarate hydratase) is an enzyme () that catalyzes the reversible hydration/dehydration of fumarate to malate. Fumarase comes in two forms: mitochondrial and cytosolic. The mitochondrial isoenzyme is involved in the Krebs cycle and the cytosolic isoenzyme is involved in the metabolism of amino acids and fumarate. Subcellular localization is established by the presence of a signal sequence on the amino terminus in the mitochondrial form, while subcellular localization in the cytosolic form is established by the absence of the signal sequence found in the mitochondrial variety.
Key facts
- Enzyme.image
- Fumarase.png
- Enzyme.name
- Fumarase
- Enzyme.EC_number
- 4.2.1.2
- Enzyme.CAS_number
- 9032-88-6
- Enzyme.GO_code
- 0004333
via Wikipedia infobox
Protein · UniProt
Fumarate hydratase, mitochondrial
- Gene
- FH
- Organism
- Homo sapiens (Human)
- Length
- 510 aa
- Molecular mass
- 54,637 Da
- Evidence
- 1: Evidence at protein level
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)
Swiss-Prot (reviewed) · via UniProt
Research
1,853 papers- Fumarate hydratase as a metabolic regulator of immunity.Trends in cell biology · 2024
- Fumarate hydratase (FH) and cancer: a paradigm of oncometabolism.British journal of cancer · 2023
- Fumarate hydratase in cancer: A multifaceted tumour suppressor.Seminars in cell & developmental biology · 2020
- Fumarate hydratase-deficient renal cell carcinoma: a case report and review of the literature.Journal of medical case reports · 2023
- Targeting metabolic and epigenetic reprogramming in metastatic fumarate hydratase-deficient renal cell carcinoma.Clinical & experimental metastasis · 2025
via PubMed
Clinical Trials
7 registered- PHASE1COMPLETEDStudy of the Glutaminase Inhibitor CB-839 in Solid TumorsCalithera Biosciences, Inc · NCT02071862
- NARECRUITINGAn Investigation of Kidney and Urothelial Tumor Metabolism in Patients Undergoing Surgical Resection and/or BiopsyUniversity of Texas Southwestern Medical Center · NCT04623502
- PHASE2ACTIVE_NOT_RECRUITINGA Study of Nivolumab In Combination With Cabozantinib in Patients With Non-Clear Cell Renal Cell CarcinomaMemorial Sloan Kettering Cancer Center · NCT03635892
- UNKNOWNFrequency, Clinical Phenotype and Genetic Analysis of Heritable Kidney Cancer SyndromesRenJi Hospital · NCT05534854
- PHASE2COMPLETEDTalazoparib and Avelumab in Participants With Metastatic Renal Cell CarcinomaMemorial Sloan Kettering Cancer Center · NCT04068831
- PHASE2UNKNOWNSintilimab Injection Combined With Inlyta in Fumarate Hydratase- Deficient Renal Cell CarcinomaWest China Hospital · NCT04387500
Wikidata facts
- Instance of
- protein
Show 5 more facts
- found in taxon
- Homo sapiens
- cell component
- extracellular exosome
- product or material produced
- DL-malic acid
- Commons category
- Fumarase
- exact match
- www.ncbi.nlm.nih.gov/protein/P07954
via Wikidata · CC0
~8 min read
Encyclopedic overview
14 sectionsContents
- Nomenclature
- Structure
- Gene
- Protein
- Subtypes
- Function
- Mechanism
- Biochemical pathway
- Other substrates
- Clinical significance
- Interactive pathway map
- See also
- References
- External links
Fumarase (or fumarate hydratase) is an enzyme () that catalyzes the reversible hydration/dehydration of fumarate to malate. Fumarase comes in two forms: mitochondrial and cytosolic. The mitochondrial isoenzyme is involved in the Krebs cycle and the cytosolic isoenzyme is involved in the metabolism of amino acids and fumarate. Subcellular localization is established by the presence of a signal sequence on the amino terminus in the mitochondrial form, while subcellular localization in the cytosolic form is established by the absence of the signal sequence found in the mitochondrial variety.
This enzyme participates in 2 metabolic pathways: citric acid cycle and reductive citric acid cycle (CO2 fixation), and is also important in renal cell carcinoma. Mutations in this gene have been associated with the development of leiomyomas in the skin and uterus in combination with renal cell carcinoma (HLRCC syndrome).
Excerpted from Wikipedia’s “fumarate hydratase” article, available under the CC BY-SA 4.0 licence.