Hydroxyacid-oxoacid transhydrogenase, mitochondrial (HOT) (EC 1.1.99.24) (Alcohol dehydrogenase iron-containing protein 1) (ADHFe1) (Fe-containing alcohol dehydrogenase)
1_MAAAA 6_ RARVA 11_ YLLRQ 16_ LQRAA 21_ CQCPT 26_ HSHTY 31_ SQAPG 36_ LSPSG 41_ KTTDY 46_ AFEMA 51_ VSNIR 56_ YGAAV 61_ TKEVG 66_ MDLKN 71_ MGAKN 76_ VCLMT 81_ DKNLS 86_ KLPPV 91_ QVAMD 96_ SLVKN 101_ GIPFT 106_ VYDNV 111_ RVEPT 116_ DSSFM 121_ EAIEF 126_ AQKGA 131_ FDAYV 136_ AVGGG 141_ STMDT 146_ CKAAN 151_ LYASS 156_ PHSDF 161_ LDYVS 166_ APIGK 171_ GKPVS 176_ VPLKP 181_ LIAVP 186_ TTSGT 191_ GSETT 196_ GVAIF 201_ DYEHL 206_ KVKIG 211_ ITSRA 216_ IKPTL 221_ GLIDP 226_ LHTLH 231_ MPARV 236_ VANSG 241_ FDVLC 246_ HALES 251_ YTTLP 256_ YHLRS 261_ PCPSN 266_ PITRP 271_ AYQGS 276_ NPISD 281_ IWAIH 286_ ALRIV 291_ AKYLK 296_ RAVRN 301_ PDDLE 306_ ARSHM 311_ HLASA 316_ FAGIG 321_ FGNAG 326_ VHLCH 331_ GMSYP 336_ ISGLV 341_ KMYKA 346_ KDYNV 351_ DHPLV 356_ PHGLS 361_ VVLTS 366_ PAVFT 371_ FTAQM 376_ FPERH 381_ LEMAE 386_ ILGAD 391_ TRTAR 396_ IQDAG 401_ LVLAD 406_ TLRKF 411_ LFDLD 416_ VDDGL 421_ AAVGY 426_ SKADI 431_ PALVK 436_ GTLPQ 441_ ERVTK 446_ LAPCP 451_ QSEED 456_ LAALF 461_EASMK
1: Catalyzes the cofactor-independent reversible oxidation of gamma-hydroxybutyrate (GHB) to succinic semialdehyde (SSA) coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG). D,L-3-hydroxyisobutyrate and L-3-hydroxybutyrate (L-3-OHB) are also substrates for HOT with 10-fold lower activities