Fumarate hydratase, mitochondrial (Fumarase) (HsFH) (EC 4.2.1.2)
1_MYRAL 6_ RLLAR 11_ SRPLV 16_ RAPAA 21_ ALASA 26_ PGLGG 31_ AAVPS 36_ FWPPN 41_ AARMA 46_ SQNSF 51_ RIEYD 56_ TFGEL 61_ KVPND 66_ KYYGA 71_ QTVRS 76_ TMNFK 81_ IGGVT 86_ ERMPT 91_ PVIKA 96_ FGILK 101_ RAAAE 106_ VNQDY 111_ GLDPK 116_ IANAI 121_ MKAAD 126_ EVAEG 131_ KLNDH 136_ FPLVV 141_ WQTGS 146_ GTQTN 151_ MNVNE 156_ VISNR 161_ AIEML 166_ GGELG 171_ SKIPV 176_ HPNDH 181_ VNKSQ 186_ SSNDT 191_ FPTAM 196_ HIAAA 201_ IEVHE 206_ VLLPG 211_ LQKLH 216_ DALDA 221_ KSKEF 226_ AQIIK 231_ IGRTH 236_ TQDAV 241_ PLTLG 246_ QEFSG 251_ YVQQV 256_ KYAMT 261_ RIKAA 266_ MPRIY 271_ ELAAG 276_ GTAVG 281_ TGLNT 286_ RIGFA 291_ EKVAA 296_ KVAAL 301_ TGLPF 306_ VTAPN 311_ KFEAL 316_ AAHDA 321_ LVELS 326_ GAMNT 331_ TACSL 336_ MKIAN 341_ DIRFL 346_ GSGPR 351_ SGLGE 356_ LILPE 361_ NEPGS 366_ SIMPG 371_ KVNPT 376_ QCEAM 381_ TMVAA 386_ QVMGN 391_ HVAVT 396_ VGGSN 401_ GHFEL 406_ NVFKP 411_ MMIKN 416_ VLHSA 421_ RLLGD 426_ ASVSF 431_ TENCV 436_ VGIQA 441_ NTERI 446_ NKLMN 451_ ESLML 456_ VTALN 461_ PHIGY 466_ DKAAK 471_ IAKTA 476_ HKNGS 481_ TLKET 486_ AIELG 491_ YLTAE 496_ QFDEW 501_VKPKD
1: Catalyzes the reversible stereospecific interconversion of fumarate to L-malate (PubMed:30761759). Experiments in other species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other (Probable)
2: Catalyzes the hydration of fumarate to L-malate in the tricarboxylic acid (TCA) cycle to facilitate a transition step in the production of energy in the form of NADH
3: Catalyzes the dehydration of L-malate to fumarate (By similarity). Fumarate metabolism in the cytosol plays a role during urea cycle and arginine metabolism; fumarate being a by-product of the urea cycle and amino-acid catabolism (By similarity). Also plays a role in DNA repair by promoting non-homologous end-joining (NHEJ) (PubMed:20231875, PubMed:26237645). In response to DNA damage and phosphorylation by PRKDC, translocates to the nucleus and accumulates at DNA double-strand breaks (DSBs): acts by catalyzing formation of fumarate, an inhibitor of KDM2B histone demethylase activity, resulting in enhanced dimethylation of histone H3 'Lys-36' (H3K36me2) (PubMed:26237645)