Carnitine O-palmitoyltransferase 1, liver isoform (CPT1-L) (EC 2.3.1.21) (Carnitine O-palmitoyltransferase I, liver isoform) (CPT I) (CPTI-L) (Carnitine palmitoyltransferase 1A)
1_MAEAH 6_ QAVAF 11_ QFTVT 16_ PDGID 21_ LRLSH 26_ EALRQ 31_ IYLSG 36_ LHSWK 41_ KKFIR 46_ FKNGI 51_ ITGVY 56_ PASPS 61_ SWLIV 66_ VVGVM 71_ TTMYA 76_ KIDPS 81_ LGIIA 86_ KINRT 91_ LETAN 96_ CMSSQ 101_ TKNVV 106_ SGVLF 111_ GTGLW 116_ VALIV 121_ TMRYS 126_ LKVLL 131_ SYHGW 136_ MFTEH 141_ GKMSR 146_ ATKIW 151_ MGMVK 156_ IFSGR 161_ KPMLY 166_ SFQTS 171_ LPRLP 176_ VPAVK 181_ DTVNR 186_ YLQSV 191_ RPLMK 196_ EEDFK 201_ RMTAL 206_ AQDFA 211_ VGLGP 216_ RLQWY 221_ LKLKS 226_ WWATN 231_ YVSDW 236_ WEEYI 241_ YLRGR 246_ GPLMV 251_ NSNYY 256_ AMDLL 261_ YILPT 266_ HIQAA 271_ RAGNA 276_ IHAIL 281_ LYRRK 286_ LDREE 291_ IKPIR 296_ LLGST 301_ IPLCS 306_ AQWER 311_ MFNTS 316_ RIPGE 321_ ETDTI 326_ QHMRD 331_ SKHIV 336_ VYHRG 341_ RYFKV 346_ WLYHD 351_ GRLLK 356_ PREME 361_ QQMQR 366_ ILDNT 371_ SEPQP 376_ GEARL 381_ AALTA 386_ GDRVP 391_ WARCR 396_ QAYFG 401_ RGKNK 406_ QSLDA 411_ VEKAA 416_ FFVTL 421_ DETEE 426_ GYRSE 431_ DPDTS 436_ MDSYA 441_ KSLLH 446_ GRCYD 451_ RWFDK 456_ SFTFV 461_ VFKNG 466_ KMGLN 471_ AEHSW 476_ ADAPI 481_ VAHLW 486_ EYVMS 491_ IDSLQ 496_ LGYAE 501_ DGHCK 506_ GDINP 511_ NIPYP 516_ TRLQW 521_ DIPGE 526_ CQEVI 531_ ETSLN 536_ TANLL 541_ ANDVD 546_ FHSFP 551_ FVAFG 556_ KGIIK 561_ KCRTS 566_ PDAFV 571_ QLALQ 576_ LAHYK 581_ DMGKF 586_ CLTYE 591_ ASMTR 596_ LFREG 601_ RTETV 606_ RSCTT 611_ ESCDF 616_ VRAMV 621_ DPAQT 626_ VEQRL 631_ KLFKL 636_ ASEKH 641_ QHMYR 646_ LAMTG 651_ SGIDR 656_ HLFCL 661_ YVVSK 666_ YLAVE 671_ SPFLK 676_ EVLSE 681_ PWRLS 686_ TSQTP 691_ QQQVE 696_ LFDLE 701_ NNPEY 706_ VSSGG 711_ GFGPV 716_ ADDGY 721_ GVSYI 726_ LVGEN 731_ LINFH 736_ ISSKF 741_ SCPET 746_ DSHRF 751_ GRHLK 756_ EAMTD 761_ IITLF 766_GLSSN
1: Catalyzes the transfer of the acyl group of long-chain fatty acid-CoA conjugates onto carnitine, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation in the mitochondrion (PubMed:11350182, PubMed:14517221, PubMed:16651524, PubMed:9691089). Also possesses a lysine succinyltransferase activity that can regulate enzymatic activity of substrate proteins such as ENO1 and metabolism independent of its classical carnitine O-palmitoyltransferase activity (PubMed:29425493). Plays an important role in hepatic triglyceride metabolism (By similarity). Also plays a role in inducible regulatory T-cell (iTreg) differentiation once activated by butyryl-CoA that antagonizes malonyl-CoA-mediated CPT1A repression (By similarity). Sustains the IFN-I response by recruiting ZDHCC4 to palmitoylate MAVS at the mitochondria leading to MAVS stabilization and activation (PubMed:38016475). Promotes ROS-induced oxidative stress in liver injury via modulation of NFE2L2 and NLRP3-mediated signaling pathways (By similarity)