Enoyl-[acyl-carrier-protein] reductase, mitochondrial (EC 1.3.1.104) (2-enoyl thioester reductase) (Nuclear receptor-binding factor 1) (HsNrbf-1) (NRBF-1)
1_MWVCS 6_ TLWRV 11_ RTPAR 16_ QWRGL 21_ LPASG 26_ CHGPA 31_ ASSYS 36_ ASAEP 41_ ARVRA 46_ LVYGH 51_ HGDPA 56_ KVVEL 61_ KNLEL 66_ AAVRG 71_ SDVRV 76_ KMLAA 81_ PINPS 86_ DINMI 91_ QGNYG 96_ FLPEL 101_ PAVGG 106_ NEGVA 111_ QVVAV 116_ GSNVT 121_ GLKPG 126_ DWVIP 131_ ANAGL 136_ GTWRT 141_ EAVFS 146_ EEALI 151_ QVPSD 156_ IPLQS 161_ AATLG 166_ VNPCT 171_ AYRML 176_ MDFEQ 181_ LQPGD 186_ SVIQN 191_ ASNSG 196_ VGQAV 201_ IQIAA 206_ ALGLR 211_ TINVV 216_ RDRPD 221_ IQKLS 226_ DRLKS 231_ LGAEH 236_ VITEE 241_ ELRRP 246_ EMKNF 251_ FKDMP 256_ QPRLA 261_ LNCVG 266_ GKSST 271_ ELLRQ 276_ LARGG 281_ TMVTY 286_ GGMAK 291_ QPVVA 296_ SVSLL 301_ IFKDL 306_ KLRGF 311_ WLSQW 316_ KKDHS 321_ PDQFK 326_ ELILT 331_ LCDLI 336_ RRGQL 341_ TAPAC 346_ SQVPL 351_ QDYQS 356_ ALEAS 361_ MKPFI 366_SSKQI
1: Catalyzes the NADPH-dependent reduction of trans-2-enoyl thioesters in mitochondrial fatty acid synthesis (fatty acid synthesis type II). Fatty acid chain elongation in mitochondria uses acyl carrier protein (ACP) as an acyl group carrier, but the enzyme accepts both ACP and CoA thioesters as substrates in vitro. Displays a preference for medium-chain over short- and long-chain substrates (PubMed:12654921, PubMed:18479707, PubMed:27817865). May provide the octanoyl chain used for lipoic acid biosynthesis, regulating protein lipoylation and mitochondrial respiratory activity particularly in Purkinje cells (By similarity). Involved in iron homeostasis; affecting Fe-S cluster assembly and ceramide metabolism (PubMed:37653044). Required for proper morphology and bioenergetic functions of mitochondria (PubMed:37653044). Required for maintenance of neurons (By similarity)