AFG3-like protein 2 (EC 3.4.24.-) (Paraplegin-like protein)
1_MAHRC 6_ LRLWG 11_ RGGCW 16_ PRGLQ 21_ QLLVP 26_ GGVGP 31_ GEQPC 36_ LRTLY 41_ RFVTT 46_ QARAS 51_ RNSLL 56_ TDIIA 61_ AYQRF 66_ CSRPP 71_ KGFEK 76_ YFPNG 81_ KNGKK 86_ ASEPK 91_ EVMGE 96_ KKESK 101_ PAATT 106_ RSSGG 111_ GGGGG 116_ GKRGG 121_ KKDDS 126_ HWWSR 131_ FQKGD 136_ IPWDD 141_ KDFRM 146_ FFLWT 151_ ALFWG 156_ GVMFY 161_ LLLKR 166_ SGREI 171_ TWKDF 176_ VNNYL 181_ SKGVV 186_ DRLEV 191_ VNKRF 196_ VRVTF 201_ TPGKT 206_ PVDGQ 211_ YVWFN 216_ IGSVD 221_ TFERN 226_ LETLQ 231_ QELGI 236_ EGENR 241_ VPVVY 246_ IAESD 251_ GSFLL 256_ SMLPT 261_ VLIIA 266_ FLLYT 271_ IRRGP 276_ AGIGR 281_ TGRGM 286_ GGLFS 291_ VGETT 296_ AKVLK 301_ DEIDV 306_ KFKDV 311_ AGCEE 316_ AKLEI 321_ MEFVN 326_ FLKNP 331_ KQYQD 336_ LGAKI 341_ PKGAI 346_ LTGPP 351_ GTGKT 356_ LLAKA 361_ TAGEA 366_ NVPFI 371_ TVSGS 376_ EFLEM 381_ FVGVG 386_ PARVR 391_ DLFAL 396_ ARKNA 401_ PCILF 406_ IDEID 411_ AVGRK 416_ RGRGN 421_ FGGQS 426_ EQENT 431_ LNQLL 436_ VEMDG 441_ FNTTT 446_ NVVIL 451_ AGTNR 456_ PDILD 461_ PALLR 466_ PGRFD 471_ RQIFI 476_ GPPDI 481_ KGRAS 486_ IFKVH 491_ LRPLK 496_ LDSTL 501_ EKDKL 506_ ARKLA 511_ SLTPG 516_ FSGAD 521_ VANVC 526_ NEAAL 531_ IAARH 536_ LSDSI 541_ NQKHF 546_ EQAIE 551_ RVIGG 556_ LEKKT 561_ QVLQP 566_ EEKKT 571_ VAYHE 576_ AGHAV 581_ AGWYL 586_ EHADP 591_ LLKVS 596_ IIPRG 601_ KGLGY 606_ AQYLP 611_ KEQYL 616_ YTKEQ 621_ LLDRM 626_ CMTLG 631_ GRVSE 636_ EIFFG 641_ RITTG 646_ AQDDL 651_ RKVTQ 656_ SAYAQ 661_ IVQFG 666_ MNEKV 671_ GQISF 676_ DLPRQ 681_ GDMVL 686_ EKPYS 691_ EATAR 696_ LIDDE 701_ VRILI 706_ NDAYK 711_ RTVAL 716_ LTEKK 721_ ADVEK 726_ VALLL 731_ LEKEV 736_ LDKND 741_ MVELL 746_ GPRPF 751_ AEKST 756_ YEEFV 761_ EGTGS 766_ LDEDT 771_ SLPEG 776_ LKDWN 781_ KEREK 786_ EKEEP 791_PGEKV
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:19748354, PubMed:28396416, PubMed:29932645, PubMed:30683687, PubMed:31327635, PubMed:37917749, PubMed:38157846). AFG3L2 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 (PubMed:19748354, PubMed:31327635). The m-AAA protease carries out quality control in the inner membrane of the mitochondria by mediating degradation of mistranslated or misfolded polypeptides (PubMed:26504172, PubMed:30683687, PubMed:34718584). The m-AAA protease complex also promotes the processing and maturation of mitochondrial proteins, such as MRPL32/bL32m, PINK1 and SP7 (PubMed:22354088, PubMed:29932645, PubMed:30252181). 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 (PubMed:29932645). Required for SPG7 maturation into its active mature form after SPG7 cleavage by mitochondrial-processing peptidase (MPP) (PubMed:30252181). Required for the maturation of PINK1 into its 52kDa mature form after its cleavage by mitochondrial-processing peptidase (MPP) (PubMed:22354088). 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:27642048, PubMed:28396416). Promotes the proteolytic degradation of GHITM upon hyperpolarization of mitochondria: progressive GHITM degradation leads to respiratory complex I degradation and broad reshaping of the mitochondrial proteome by AFG3L2 (PubMed:35912435). Also acts as a regulator of mitochondrial glutathione homeostasis by mediating cleavage and degradation of SLC25A39 (PubMed:37917749, PubMed:38157846). SLC25A39 cleavage is prevented when SLC25A39 binds iron-sulfur (PubMed:37917749, PubMed:38157846). Involved in the regulation of OMA1-dependent processing of OPA1 (PubMed:17615298, PubMed:29545505, PubMed:30252181, PubMed:30683687, PubMed:32600459). May act by mediating processing of OMA1 precursor, participating in OMA1 maturation (PubMed:29545505)