Fumarase

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fumarate hydratase
Identifiers
Symbol	FH
Entrez	2271
HUGO	3700
OMIM	136850
RefSeq	NM_000143
UniProt	P07954
Other data
EC number	4.2.1.2
Locus	Chr. 1 q42.1

Fumarase (or fumarate hydratase) is an enzyme involved in the Krebs Cycle (aka the Tricarboxylic Acid Cycle aka the Citric Acid Cycle) that catalyzes the hydration (addition of H₂O across a double bond) of Fumarate to L-malate (See the entry on optical isomer).

Fumarate + H2O <-> L Malate

[edit] Biochemical Pathway

• Diagram TCA Cycle at KEGG, gives some details on the TCA cycle in which fumerase acts. [Fumarase is labeled '4.2.1.2' in the lower left hand corner of this diagram.

• structure of Fumarate
• Structure of L Malate

• See also Citric Acid Cycle on Wikipedia.

[edit] See also

• Fumarase deficiency

[edit] External links

• Crystal structure of Yeast Fumarase at the Protein Data Bank.
• MeSH Fumarase

v • d • e Carbon-oxygen lyases (EC 4.2) (primarily dehydratases) Carbonic anhydrase - Fumarase - Aconitase - Enolase (Alpha) - Enoyl-CoA hydratase/3-Hydroxyacyl ACP dehydrase - Methylglutaconyl-CoA hydratase - Tryptophan synthase - Cystathionine beta synthase - Porphobilinogen synthase - 3-isopropylmalate dehydratase - Urocanate hydratase - Uroporphyrinogen III synthase - Nitrile hydratase

v • d • e Metabolism: Citric acid cycle enzymes Cycle Citrate synthase - Aconitase - Isocitrate dehydrogenase - Oxoglutarate dehydrogenase - Succinyl CoA synthetase Succinate dehydrogenase (SDHA) - Fumarase - Malate dehydrogenase Anaplerotic to acetyl-CoA: Pyruvate dehydrogenase complex (regulated by Pyruvate dehydrogenase kinase and Pyruvate dehydrogenase phosphatase) to ketoglutaric acid: Glutamate dehydrogenase to succinyl-CoA: Methylmalonyl-CoA mutase to oxaloacetate: Pyruvate carboxylase - Aspartate transaminase

v • d • e Mitochondrial enzymes and transporters Outer membrane fatty acid degradation (Carnitine palmitoyltransferase I, Long fatty acyl CoA synthetase) tryptophan metabolism (Kynureninase) monoamine neurotransmitter metabolism (Monoamine oxidase) Intermembrane space Adenylate kinase - Creatine kinase Inner membrane oxidative phosphorylation (Coenzyme Q - cytochrome c reductase, Cytochrome c, NADH dehydrogenase, Succinate dehydrogenase) pyrimidine metabolism (Dihydroorotate dehydrogenase) mitochondrial shuttle (Malate-aspartate shuttle, Glycerol phosphate shuttle) other (Glutamate aspartate transporter, Glycerol-3-phosphate dehydrogenase, ATP synthase, Carnitine palmitoyltransferase II) Matrix citric acid cycle (Citrate synthase, Aconitase, Isocitrate dehydrogenase, Oxoglutarate dehydrogenase, Succinyl coenzyme A synthetase, Fumarase, Malate dehydrogenase) anaplerotic reactions (Aspartate transaminase, Glutamate dehydrogenase, Pyruvate dehydrogenase complex) urea cycle (Carbamoyl phosphate synthetase I, Ornithine transcarbamylase, N-Acetylglutamate synthase) alcohol metabolism (ALDH2) Mitochondrial DNA Complex I (7 units) - Complex III (1 unit) - Complex IV (3 units) - ATP synthase (2 units)