Lon protease homolog, mitochondrial (EC 3.4.21.53) (LONHs) (Lon protease-like protein) (LONP) (Mitochondrial ATP-dependent protease Lon) (Serine protease 15)
1_MAAST 6_ GYVRL 11_ WGAAR 16_ CWVLR 21_ RPMLA 26_ AAGGR 31_ VPTAA 36_ GAWLL 41_ RGQRT 46_ CDASP 51_ PWALW 56_ GRGPA 61_ IGGQW 66_ RGFWE 71_ ASSRG 76_ GGAFS 81_ GGEDA 86_ SEGGA 91_ EEGAG 96_ GAGGS 101_ AGAGE 106_ GPVIT 111_ ALTPM 116_ TIPDV 121_ FPHLP 126_ LIAIT 131_ RNPVF 136_ PRFIK 141_ IIEVK 146_ NKKLV 151_ ELLRR 156_ KVRLA 161_ QPYVG 166_ VFLKR 171_ DDSNE 176_ SDVVE 181_ SLDEI 186_ YHTGT 191_ FAQIH 196_ EMQDL 201_ GDKLR 206_ MIVMG 211_ HRRVH 216_ ISRQL 221_ EVEPE 226_ EPEAE 231_ NKHKP 236_ RRKSK 241_ RGKKE 246_ AEDEL 251_ SARHP 256_ AELAM 261_ EPTPE 266_ LPAEV 271_ LMVEV 276_ ENVVH 281_ EDFQV 286_ TEEVK 291_ ALTAE 296_ IVKTI 301_ RDIIA 306_ LNPLY 311_ RESVL 316_ QMMQA 321_ GQRVV 326_ DNPIY 331_ LSDMG 336_ AALTG 341_ AESHE 346_ LQDVL 351_ EETNI 356_ PKRLY 361_ KALSL 366_ LKKEF 371_ ELSKL 376_ QQRLG 381_ REVEE 386_ KIKQT 391_ HRKYL 396_ LQEQL 401_ KIIKK 406_ ELGLE 411_ KDDKD 416_ AIEEK 421_ FRERL 426_ KELVV 431_ PKHVM 436_ DVVDE 441_ ELSKL 446_ GLLDN 451_ HSSEF 456_ NVTRN 461_ YLDWL 466_ TSIPW 471_ GKYSN 476_ ENLDL 481_ ARAQA 486_ VLEED 491_ HYGME 496_ DVKKR 501_ ILEFI 506_ AVSQL 511_ RGSTQ 516_ GKILC 521_ FYGPP 526_ GVGKT 531_ SIARS 536_ IARAL 541_ NREYF 546_ RFSVG 551_ GMTDV 556_ AEIKG 561_ HRRTY 566_ VGAMP 571_ GKIIQ 576_ CLKKT 581_ KTENP 586_ LILID 591_ EVDKI 596_ GRGYQ 601_ GDPSS 606_ ALLEL 611_ LDPEQ 616_ NANFL 621_ DHYLD 626_ VPVDL 631_ SKVLF 636_ ICTAN 641_ VTDTI 646_ PEPLR 651_ DRMEM 656_ INVSG 661_ YVAQE 666_ KLAIA 671_ ERYLV 676_ PQARA 681_ LCGLD 686_ ESKAK 691_ LSSDV 696_ LTLLI 701_ KQYCR 706_ ESGVR 711_ NLQKQ 716_ VEKVL 721_ RKSAY 726_ KIVSG 731_ EAESV 736_ EVTPE 741_ NLQDF 746_ VGKPV 751_ FTVER 756_ MYDVT 761_ PPGVV 766_ MGLAW 771_ TAMGG 776_ STLFV 781_ ETSLR 786_ RPQDK 791_ DAKGD 796_ KDGSL 801_ EVTGQ 806_ LGEVM 811_ KESAR 816_ IAYTF 821_ ARAFL 826_ MQHAP 831_ ANDYL 836_ VTSHI 841_ HLHVP 846_ EGATP 851_ KDGPS 856_ AGCTI 861_ VTALL 866_ SLAMG 871_ RPVRQ 876_ NLAMT 881_ GEVSL 886_ TGKIL 891_ PVGGI 896_ KEKTI 901_ AAKRA 906_ GVTCI 911_ VLPAE 916_ NKKDF 921_ YDLAA 926_ FITEG 931_ LEVHF 936_ VEHYR 941_ EIFDI 946_ AFPDE 951_QAEAL
1: ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix (PubMed:12198491, PubMed:15870080, PubMed:17579211, PubMed:37327776, PubMed:8248235). Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein, DELE1, helicase Twinkle (TWNK) and the large ribosomal subunit protein MRPL32/bL32m (PubMed:17579211, PubMed:28377575, PubMed:37327776). MRPL32/bL32m is protected from degradation by LONP1 when it is bound to a nucleic acid (RNA), but TWNK is not (PubMed:17579211, PubMed:28377575). May also have a chaperone function in the assembly of inner membrane protein complexes (By similarity). Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome (PubMed:17420247). Binds to mitochondrial promoters and RNA in a single-stranded, site-specific, and strand-specific manner (PubMed:17420247). May regulate mitochondrial DNA replication and/or gene expression using site-specific, single-stranded DNA binding to target the degradation of regulatory proteins binding to adjacent sites in mitochondrial promoters (PubMed:14739292, PubMed:17420247)