Protein: Fumarate hydratase, mitochondrial (Fumarase) (HsFH) (EC 4.2.1.2) (P07954)
Organism: Homo sapiens (Human)
Function: Catalyzes the reversible stereospecific interconversion of fumarate to L-malate. Experiments in other species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other (Probable).
Subcellular location: [Isoform Mitochondrial]: Mitochondrion {ECO:0000269|PubMed:27037871}.; SUBCELLULAR LOCATION: [Isoform Cytoplasmic]: Cytoplasm, cytosol {ECO:0000269|PubMed:20231875, ECO:0000269|PubMed:22509282, ECO:0000269|PubMed:26237645, ECO:0000269|PubMed:27037871}. Nucleus {ECO:0000269|PubMed:20231875, ECO:0000269|PubMed:26237645}. Chromosome {ECO:0000269|PubMed:26237645}. Note=Translocates to the nucleus in response to DNA damage: localizes to DNA double-strand breaks (DSBs) following phosphorylation by PRKDC. {ECO:0000269|PubMed:26237645}.
Sequence: MYRALRLLARSRPLVRAPAAALASAPGLGGAAVPSFWPPNAARMASQNSFRIEYDTFGELKVPNDKYYGAQTVRSTMNFKIGGVTERMPTPVIKAFGILKRAAAEVNQDYGLDPKIANAIMKAADEVAEGKLNDHFPLVVWQTGSGTQTNMNVNEVISNRAIEMLGGELGSKIPVHPNDHVNKSQSSNDTFPTAMHIAAAIEVHEVLLPGLQKLHDALDAKSKEFAQIIKIGRTHTQDAVPLTLGQEFSGYVQQVKYAMTRIKAAMPRIYELAAGGTAVGTGLNTRIGFAEKVAAKVAALTGLPFVTAPNKFEALAAHDALVELSGAMNTTACSLMKIANDIRFLGSGPRSGLGELILPENEPGSSIMPGKVNPTQCEAMTMVAAQVMGNHVAVTVGGSNGHFELNVFKPMMIKNVLHSARLLGDASVSFTENCVVGIQANTERINKLMNESLMLVTALNPHIGYDKAAKIAKTAHKNGSTLKETAIELGYLTAEQFDEWVKPKDMLGPK
InterPro domains: - IPR008948: L-Aspartase-like (homologous_superfamily) [48-503]
- IPR018951: Fumarase C, C-terminal (domain) [455-507]
- IPR000362: Fumarate lyase family (family) [181-380]
- IPR020557: Fumarate lyase, conserved site (conserved_site) [364-373]
- IPR022761: Fumarate lyase, N-terminal (domain) [58-389]
- IPR005677: Fumarate hydratase, class II (family) [40-510]
- IPR024083: Fumarase/histidase, N-terminal (homologous_superfamily) [51-185]
Interaction partners: - Succinate dehydrogenase flavoprotein subunit, mitochondrial
- Succinate dehydrogenase iron-sulfur subunit, mitochondrial
- Malate dehydrogenase, mitochondrial
- Succinate dehydrogenase cytochrome b560 subunit, mitochondrial
- Succinate dehydrogenase cytochrome b small subunit, mitochondrial
- Citrate synthase, mitochondrial
- NADP-dependent malic enzyme
- NAD-dependent malic enzyme, mitochondrial
- NADP-dependent malic enzyme, mitochondrial
- Aspartate aminotransferase, mitochondrial
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0003824 (catalytic activity), GO:0005515 (protein binding), GO:0016829 (lyase activity), GO:0016835 (carbon-oxygen lyase activity), GO:0016836 (hydro-lyase activity)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0009987 (cellular process), GO:0044237 (cellular metabolic process), GO:0071704 (organic substance metabolic process), GO:0044281 (small molecule metabolic process), GO:0006082 (organic acid metabolic process), GO:0043436 (oxoacid metabolic process), GO:0019752 (carboxylic acid metabolic process), GO:0043648 (dicarboxylic acid metabolic process), GO:0006106 (fumarate metabolic process), GO:0006108 (malate metabolic process)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0005737 (cytoplasm), GO:0043229 (intracellular organelle), GO:0005739 (mitochondrion), GO:0043227 (membrane-bounded organelle), GO:0043231 (intracellular membrane-bounded organelle)