Paraplegin (EC 3.4.24.-) (Cell matrix adhesion regulator) (Spastic paraplegia 7 protein)
1_MAVLL 6_ LLLRA 11_ LRRGP 16_ GPGPR 21_ PLWGP 26_ GPAWS 31_ PGFPA 36_ RPGRG 41_ RPYMA 46_ SRPPG 51_ DLAEA 56_ GGRAL 61_ QSLQL 66_ RLLTP 71_ TFEGI 76_ NGLLL 81_ KQHLV 86_ QNPVR 91_ LWQLL 96_ GGTFY 101_ FNTSR 106_ LKQKN 111_ KEKDK 116_ SKGKA 121_ PEEDE 126_ EERRR 131_ RERDD 136_ QMYRE 141_ RLRTL 146_ LVIAV 151_ VMSLL 156_ NALST 161_ SGGSI 166_ SWNDF 171_ VHEML 176_ AKGEV 181_ QRVQV 186_ VPESD 191_ VVEVY 196_ LHPGA 201_ VVFGR 206_ PRLAL 211_ MYRMQ 216_ VANID 221_ KFEEK 226_ LRAAE 231_ DELNI 236_ EAKDR 241_ IPVSY 246_ KRTGF 251_ FGNAL 256_ YSVGM 261_ TAVGL 266_ AILWY 271_ VFRLA 276_ GMTGR 281_ EGGFS 286_ AFNQL 291_ KMARF 296_ TIVDG 301_ KMGKG 306_ VSFKD 311_ VAGMH 316_ EAKLE 321_ VREFV 326_ DYLKS 331_ PERFL 336_ QLGAK 341_ VPKGA 346_ LLLGP 351_ PGCGK 356_ TLLAK 361_ AVATE 366_ AQVPF 371_ LAMAG 376_ PEFVE 381_ VIGGL 386_ GAARV 391_ RSLFK 396_ EARAR 401_ APCIV 406_ YIDEI 411_ DAVGK 416_ KRSTT 421_ MSGFS 426_ NTEEE 431_ QTLNQ 436_ LLVEM 441_ DGMGT 446_ TDHVI 451_ VLAST 456_ NRADI 461_ LDGAL 466_ MRPGR 471_ LDRHV 476_ FIDLP 481_ TLQER 486_ REIFE 491_ QHLKS 496_ LKLTQ 501_ SSTFY 506_ SQRLA 511_ ELTPG 516_ FSGAD 521_ IANIC 526_ NEAAL 531_ HAARE 536_ GHTSV 541_ HTLNF 546_ EYAVE 551_ RVLAG 556_ TAKKS 561_ KILSK 566_ EEQKV 571_ VAFHE 576_ SGHAL 581_ VGWML 586_ EHTEA 591_ VMKVS 596_ ITPRT 601_ NAALG 606_ FAQML 611_ PRDQH 616_ LFTKE 621_ QLFER 626_ MCMAL 631_ GGRAS 636_ EALSF 641_ NEVTS 646_ GAQDD 651_ LRKVT 656_ RIAYS 661_ MVKQF 666_ GMAPG 671_ IGPIS 676_ FPEAQ 681_ EGLMG 686_ IGRRP 691_ FSQGL 696_ QQMMD 701_ HEARL 706_ LVAKA 711_ YRHTE 716_ KVLQD 721_ NLDKL 726_ QALAN 731_ ALLEK 736_ EVINY 741_ EDIEA 746_ LIGPP 751_ PHGPK 756_ KMIAP 761_ QRWID 766_ AQREK 771_ QDLGE 776_ EETEE 781_ TQQPP 786_LGGEE
1: Catalytic component of the m-AAA protease, a protease that plays a key role in proteostasis of inner mitochondrial membrane proteins, and which is essential for axonal and neuron development (PubMed:11549317, PubMed:28396416, PubMed:31097542, PubMed:9635427). SPG7 possesses both ATPase and protease activities: the ATPase activity is required to unfold substrates, threading them into the internal proteolytic cavity for hydrolysis into small peptide fragments (By similarity). The m-AAA protease exerts a dual role in the mitochondrial inner membrane: it mediates the processing of specific regulatory proteins and ensures protein quality control by degrading misfolded polypeptides (By similarity). Mediates protein maturation of the mitochondrial ribosomal subunit MRPL32/bL32m by catalyzing the cleavage of the presequence of MRPL32/bL32m prior to assembly into the mitochondrial ribosome (By similarity). Acts as a regulator of calcium in neurons by mediating degradation of SMDT1/EMRE before its assembly with the uniporter complex, limiting the availability of SMDT1/EMRE for MCU assembly and promoting efficient assembly of gatekeeper subunits with MCU (PubMed:28396416, PubMed:31097542). Also regulates mitochondrial calcium by catalyzing degradation of MCU (PubMed:31097542). Plays a role in the formation and regulation of the mitochondrial permeability transition pore (mPTP) and its proteolytic activity is dispensable for this function (PubMed:26387735)