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Fumarase 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.

FH
Available structures
PDB Ortholog search: PDBe RCSB
Identifiers
Aliases FH, fumarate hydratase, HLRCC, LRCC, MCL, MCUL1, FMRD
External IDs MGI: 95530 HomoloGene: 115 GeneCards: FH
Orthologs
Species Human Mouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_000143

NM_010209

RefSeq (protein)

NP_000134.2

NP_034339.2

Location (UCSC) Chr 1: 241.5 – 241.52 Mb Chr 1: 175.6 – 175.63 Mb
PubMed search
Wikidata
View/Edit Human View/Edit Mouse

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 (also known as the Tricarboxylic Acid Cycle [TCA] or the Citric Acid 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, 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.

Extra Credit-Biochemistry-Fumarase Deficiency

This video is about what goes wrong in the citric acid cycle with fumarase deficiency.

Fumarase Deficiency

CREDITS https://ghr.nlm.nih.gov/condition/fumarase-deficiency https://ghr.nlm.nih.gov/gene/FH# Mullins, KJ. "Mormon Sect's Polygamy Causes Most of the ...

Fumarase Meaning

Video shows what fumarase means. An enzyme that in the Krebs cycle acts as a catalyst for the reaction of water and fumarate to form L-malate.. Fumarase ...

Fumarase deficiency (Medical Condition)

Symptoms, risk factors and treatments of Fumarase deficiency (Medical Condition) Fumarase deficiency, also known as "Polygamist Down's", is an autosomal ...

Polygamy Town: Colorado City and it's recent turn of events

Colorado City, Arizona it is one of the most infamous towns in all of Arizona. But beneath its beauty and locale lies a dark history. It's home to hundreds of ...

Raising Micah

A documentary of my nephew with Fumarase Deficiency.