Trifunctional enzyme subunit alpha, mitochondrial (78 kDa gastrin-binding protein) (Monolysocardiolipin acyltransferase) (EC 2.3.1.-) (TP-alpha) [Includes: Long-chain enoyl-CoA hydratase (EC 4.2.1.17); Long chain 3-hydroxyacyl-CoA dehydrogenase (EC 1.1.1.211)]
1_MVACR 6_ AIGIL 11_ SRFSA 16_ FRILR 21_ SRGYI 26_ CRNFT 31_ GSSAL 36_ LTRTH 41_ INYGV 46_ KGDVA 51_ VVRIN 56_ SPNSK 61_ VNTLS 66_ KELHS 71_ EFSEV 76_ MNEIW 81_ ASDQI 86_ RSAVL 91_ ISSKP 96_ GCFIA 101_ GADIN 106_ MLAAC 111_ KTLQE 116_ VTQLS 121_ QEAQR 126_ IVEKL 131_ EKSTK 136_ PIVAA 141_ INGSC 146_ LGGGL 151_ EVAIS 156_ CQYRI 161_ ATKDR 166_ KTVLG 171_ TPEVL 176_ LGALP 181_ GAGGT 186_ QRLPK 191_ MVGVP 196_ AALDM 201_ MLTGR 206_ SIRAD 211_ RAKKM 216_ GLVDQ 221_ LVEPL 226_ GPGLK 231_ PPEER 236_ TIEYL 241_ EEVAI 246_ TFAKG 251_ LADKK 256_ ISPKR 261_ DKGLV 266_ EKLTA 271_ YAMTI 276_ PFVRQ 281_ QVYKK 286_ VEEKV 291_ RKQTK 296_ GLYPA 301_ PLKII 306_ DVVKT 311_ GIEQG 316_ SDAGY 321_ LCESQ 326_ KFGEL 331_ VMTKE 336_ SKALM 341_ GLYHG 346_ QVLCK 351_ KNKFG 356_ APQKD 361_ VKHLA 366_ ILGAG 371_ LMGAG 376_ IAQVS 381_ VDKGL 386_ KTILK 391_ DATLT 396_ ALDRG 401_ QQQVF 406_ KGLND 411_ KVKKK 416_ ALTSF 421_ ERDSI 426_ FSNLT 431_ GQLDY 436_ QGFEK 441_ ADMVI 446_ EAVFE 451_ DLSLK 456_ HRVLK 461_ EVEAV 466_ IPDHC 471_ IFASN 476_ TSALP 481_ ISEIA 486_ AVSKR 491_ PEKVI 496_ GMHYF 501_ SPVDK 506_ MQLLE 511_ IITTE 516_ KTSKD 521_ TSASA 526_ VAVGL 531_ KQGKV 536_ IIVVK 541_ DGPGF 546_ YTTRC 551_ LAPMM 556_ SEVIR 561_ ILQEG 566_ VDPKK 571_ LDSLT 576_ TSFGF 581_ PVGAA 586_ TLVDE 591_ VGVDV 596_ AKHVA 601_ EDLGK 606_ VFGER 611_ FGGGN 616_ PELLT 621_ QMVSK 626_ GFLGR 631_ KSGKG 636_ FYIYQ 641_ EGVKR 646_ KDLNS 651_ DMDSI 656_ LASLK 661_ LPPKS 666_ EVSSD 671_ EDIQF 676_ RLVTR 681_ FVNEA 686_ VMCLQ 691_ EGILA 696_ TPAEG 701_ DIGAV 706_ FGLGF 711_ PPCLG 716_ GPFRF 721_ VDLYG 726_ AQKIV 731_ DRLKK 736_ YEAAY 741_ GKQFT 746_ PCQLL 751_ ADHAN 756_SPNKK
1: Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway (PubMed:1550553, PubMed:29915090, PubMed:30850536, PubMed:8135828, PubMed:31604922). The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA (PubMed:29915090). Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids (PubMed:30850536, PubMed:31604922). Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA described here carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities while the trifunctional enzyme subunit beta/HADHB bears the 3-ketoacyl-CoA thiolase activity (PubMed:29915090, PubMed:30850536, PubMed:8135828). Independently of subunit beta, HADHA also exhibits a cardiolipin acyltransferase activity that participates in cardiolipin remodeling; cardiolipin is a major mitochondrial membrane phospholipid (PubMed:23152787, PubMed:31604922). HADHA may act downstream of Tafazzin/TAZ, that remodels monolysocardiolipin (MLCL) to a cardiolipin intermediate, and then HADHA may continue to remodel this species into mature tetralinoleoyl-cardiolipin (PubMed:31604922). Has also been proposed to act directly on MLCL; capable of acylating MLCL using different acyl-CoA substrates, with highest activity for oleoyl-CoA (PubMed:23152787)