Gene name: PITRM1

Uniprot entry:

Q5JRX3

Protein names:

Presequence protease, mitochondrial (hPreP) (EC 3.4.24.-) (Pitrilysin metalloproteinase 1) (Metalloprotease 1) (hMP1)

Protein sequence:

1_MWRCG 6_ GRQGL 11_ CVLRR 16_ LSGGH 21_ AHHRA 26_ WRWNS 31_ NRACE 36_ RALQY 41_ KLGDK 46_ IHGFT 51_ VNQVT 56_ SVPEL 61_ FLTAV 66_ KLTHD 71_ DTGAR 76_ YLHLA 81_ REDTN 86_ NLFSV 91_ QFRTT 96_ PMDST 101_ GVPHI 106_ LEHTV 111_ LCGSQ 116_ KYPCR 121_ DPFFK 126_ MLNRS 131_ LSTFM 136_ NAFTA 141_ SDYTL 146_ YPFST 151_ QNPKD 156_ FQNLL 161_ SVYLD 166_ ATFFP 171_ CLREL 176_ DFWQE 181_ GWRLE 186_ HENPS 191_ DPQTP 196_ LVFKG 201_ VVFNE 206_ MKGAF 211_ TDNER 216_ IFSQH 221_ LQNRL 226_ LPDHT 231_ YSVVS 236_ GGDPL 241_ CIPEL 246_ TWEQL 251_ KQFHA 256_ THYHP 261_ SNARF 266_ FTYGN 271_ FPLEQ 276_ HLKQI 281_ HEEAL 286_ SKFQK 291_ IEPST 296_ VVPAQ 301_ TPWDK 306_ PREFQ 311_ ITCGP 316_ DSFAT 321_ DPSKQ 326_ TTISV 331_ SFLLP 336_ DITDT 341_ FEAFT 346_ LSLLS 351_ SLLTS 356_ GPNSP 361_ FYKAL 366_ IESGL 371_ GTDFS 376_ PDVGY 381_ NGYTR 386_ EAYFS 391_ VGLQG 396_ IAEKD 401_ IETVR 406_ SLIDR 411_ TIDEV 416_ VEKGF 421_ EDDRI 426_ EALLH 431_ KIEIQ 436_ MKHQS 441_ TSFGL 446_ MLTSY 451_ IASCW 456_ NHDGD 461_ PVELL 466_ KLGNQ 471_ LAKFR 476_ QCLQE 481_ NPKFL 486_ QEKVK 491_ QYFKN 496_ NQHKL 501_ TLSMR 506_ PDDKY 511_ HEKQA 516_ QVEAT 521_ KLKQK 526_ VEALS 531_ PGDRQ 536_ QIYEK 541_ GLELR 546_ SQQSK 551_ PQDAS 556_ CLPAL 561_ KVSDI 566_ EPTIP 571_ VTELD 576_ VVLTA 581_ GDIPV 586_ QYCAQ 591_ PTNGM 596_ VYFRA 601_ FSSLN 606_ TLPEE 611_ LRPYV 616_ PLFCS 621_ VLTKL 626_ GCGLL 631_ DYREQ 636_ AQQIE 641_ LKTGG 646_ MSASP 651_ HVLPD 656_ DSHMD 661_ TYEQG 666_ VLFSS 671_ LCLDR 676_ NLPDM 681_ MQLWS 686_ EIFNN 691_ PCFEE 696_ EEHFK 701_ VLVKM 706_ TAQEL 711_ ANGIP 716_ DSGHL 721_ YASIR 726_ AGRTL 731_ TPAGD 736_ LQETF 741_ SGMDQ 746_ VRLMK 751_ RIAEM 756_ TDIKP 761_ ILRKL 766_ PRIKK 771_ HLLNG 776_ DNMRC 781_ SVNAT 786_ PQQMP 791_ QTEKA 796_ VEDFL 801_ RSIGR 806_ SKKER 811_ RPVRP 816_ HTVEK 821_ PVPSS 826_ SGGDA 831_ HVPHG 836_ SQVIR 841_ KLVME 846_ PTFKP 851_ WQMKT 856_ HFLMP 861_ FPVNY 866_ VGECI 871_ RTVPY 876_ TDPDH 881_ ASLKI 886_ LARLM 891_ TAKFL 896_ HTEIR 901_ EKGGA 906_ YGGGA 911_ KLSHN 916_ GIFTL 921_ YSYRD 926_ PNTIE 931_ TLQSF 936_ GKAVD 941_ WAKSG 946_ KFTQQ 951_ DIDEA 956_ KLSVF 961_ STVDA 966_ PVAPS 971_ DKGMD 976_ HFLYG 981_ LSDEM 986_ KQAHR 991_ EQLFA 996_ VSHDK 1001_ LLAVS 1006_ DRYLG 1011_ TGKST 1016_ HGLAI 1021_ LGPEN 1026_ PKIAK 1031_DPSWI

Protein annotations

Protein functions:

1: Metalloendopeptidase of the mitochondrial matrix that functions in peptide cleavage and degradation rather than in protein processing (PubMed:10360838, PubMed:16849325, PubMed:19196155, PubMed:24931469). Has an ATP-independent activity (PubMed:16849325). Specifically cleaves peptides in the range of 5 to 65 residues (PubMed:19196155). Shows a preference for cleavage after small polar residues and before basic residues, but without any positional preference (PubMed:10360838, PubMed:19196155, PubMed:24931469). Degrades the transit peptides of mitochondrial proteins after their cleavage (PubMed:19196155). Also degrades other unstructured peptides (PubMed:19196155). It is also able to degrade amyloid-beta protein 40, one of the peptides produced by APP processing, when it accumulates in mitochondrion (PubMed:16849325, PubMed:24931469, PubMed:26697887). It is a highly efficient protease, at least toward amyloid-beta protein 40 (PubMed:24931469, PubMed:29383861, PubMed:29764912). Cleaves that peptide at a specific position and is probably not processive, releasing digested peptides intermediates that can be further cleaved subsequently (PubMed:24931469). It is also able to degrade amyloid-beta protein 42 (PubMed:29764912)