Fumarase

FH
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 3E04
Identifiers
Aliases	FH, fumarate hydratase, HLRCC, LRCC, MCL, MCUL1, FMRD
External IDs	MGI: 95530 HomoloGene: 115 GeneCards: FH
Gene ontology Molecular function • fumarate hydratase activity • protein binding • catalytic activity • lyase activity Cellular component • cytoplasm • tricarboxylic acid cycle enzyme complex • mitochondrial matrix • extracellular exosome • mitochondrion Biological process • homeostasis of number of cells within a tissue • tricarboxylic acid cycle • malate metabolic process • fumarate metabolic process Sources:Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	2271	14194
Ensembl	ENSG00000091483	ENSMUSG00000026526
UniProt	P07954	P97807
RefSeq (mRNA)	NM_000143	NM_010209
RefSeq (protein)	NP_000134	NP_034339.2 NP_034339
Location (UCSC)	Chr 1: 241.5 – 241.52 Mb	Chr 1: 175.6 – 175.63 Mb
PubMed search

3E04

2271

14194

ENSG00000091483

ENSMUSG00000026526

P07954

P97807

NM_000143

NM_010209

NP_000134

NP_034339.2
NP_034339

Fumarase (or fumarate hydratase) is an enzyme that catalyzes the reversible hydration/dehydration of fumarate to malate. Fumarase comes in two forms: 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.

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