Gene name: EHHADH

Uniprot entry:

Q08426

Protein names:

Peroxisomal bifunctional enzyme (PBE) (PBFE) (L-bifunctional protein) (LBP) (Multifunctional enzyme 1) (MFE1) [Includes: Enoyl-CoA hydratase/3,2-trans-enoyl-CoA isomerase (EC 4.2.1.17) (EC 5.3.3.8); 3-hydroxyacyl-CoA dehydrogenase (EC 1.1.1.35)]

Protein sequence:

1_MAEYT 6_ RLHNA 11_ LALIR 16_ LRNPP 21_ VNAIS 26_ TTLLR 31_ DIKEG 36_ LQKAV 41_ IDHTI 46_ KAIVI 51_ CGAEG 56_ KFSAG 61_ ADIRG 66_ FSAPR 71_ TFGLT 76_ LGHVV 81_ DEIQR 86_ NEKPV 91_ VAAIQ 96_ GMAFG 101_ GGLEL 106_ ALGCH 111_ YRIAH 116_ AEAQV 121_ GLPEV 126_ TLGLL 131_ PGARG 136_ TQLLP 141_ RLTGV 146_ PAALD 151_ LITSG 156_ RRILA 161_ DEALK 166_ LGILD 171_ KVVNS 176_ DPVEE 181_ AIRFA 186_ QRVSD 191_ QPLES 196_ RRLCN 201_ KPIQS 206_ LPNMD 211_ SIFSE 216_ ALLKM 221_ RRQHP 226_ GCLAQ 231_ EACVR 236_ AVQAA 241_ VQYPY 246_ EVGIK 251_ KEEEL 256_ FLYLL 261_ QSGQA 266_ RALQY 271_ AFFAE 276_ RKANK 281_ WSTPS 286_ GASWK 291_ TASAR 296_ PVSSV 301_ GVVGL 306_ GTMGR 311_ GIVIS 316_ FARAR 321_ IPVIA 326_ VDSDK 331_ NQLAT 336_ ANKMI 341_ TSVLE 346_ KEASK 351_ MQQSG 356_ HPWSG 361_ PKPRL 366_ TSSVK 371_ ELGGV 376_ DLVIE 381_ AVFEE 386_ MSLKK 391_ QVFAE 396_ LSAVC 401_ KPEAF 406_ LCTNT 411_ SALDV 416_ DEIAS 421_ STDRP 426_ HLVIG 431_ THFFS 436_ PAHVM 441_ KLLEV 446_ IPSQY 451_ SSPTT 456_ IATVM 461_ NLSKK 466_ IKKIG 471_ VVVGN 476_ CFGFV 481_ GNRML 486_ NPYYN 491_ QAYFL 496_ LEEGS 501_ KPEEV 506_ DQVLE 511_ EFGFK 516_ MGPFR 521_ VSDLA 526_ GLDVG 531_ WKSRK 536_ GQGLT 541_ GPTLL 546_ PGTPA 551_ RKRGN 556_ RRYCP 561_ IPDVL 566_ CELGR 571_ FGQKT 576_ GKGWY 581_ QYDKP 586_ LGRIH 591_ KPDPW 596_ LSKFL 601_ SRYRK 606_ THHIE 611_ PRTIS 616_ QDEIL 621_ ERCLY 626_ SLINE 631_ AFRIL 636_ GEGIA 641_ ASPEH 646_ IDVVY 651_ LHGYG 656_ WPRHK 661_ GGPMF 666_ YASTV 671_ GLPTV 676_ LEKLQ 681_ KYYRQ 686_ NPDIP 691_ QLEPS 696_ DYLKK 701_ LASQG 706_ NPPLK 711_ EWQSL 716_AGSPS

Protein annotations

Protein functions:

1: Peroxisomal trifunctional enzyme possessing 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and delta 3, delta 2-enoyl-CoA isomerase activities. Catalyzes two of the four reactions of the long chain fatty acids peroxisomal beta-oxidation pathway (By similarity). Can also use branched-chain fatty acids such as 2-methyl-2E-butenoyl-CoA as a substrate, which is hydrated into (2S,3S)-3-hydroxy-2-methylbutanoyl-CoA (By similarity). Optimal isomerase for 2,5 double bonds into 3,5 form isomerization in a range of enoyl-CoA species (Probable). Also able to isomerize both 3-cis and 3-trans double bonds into the 2-trans form in a range of enoyl-CoA species (By similarity). With HSD17B4, catalyzes the hydration of trans-2-enoyl-CoA and the dehydrogenation of 3-hydroxyacyl-CoA, but with opposite chiral specificity (PubMed:15060085). Regulates the amount of medium-chain dicarboxylic fatty acids which are essential regulators of all fatty acid oxidation pathways (By similarity). Also involved in the degradation of long-chain dicarboxylic acids through peroxisomal beta-oxidation (PubMed:15060085)