fumarate hydratase
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
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.
Article
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).