Presenilins-associated rhomboid-like protein, mitochondrial (EC 3.4.21.105) (Mitochondrial intramembrane cleaving protease PARL) [Cleaved into: P-beta (Pbeta)]
1_MAWRG 6_ WAQRG 11_ WGCGQ 16_ AWGAS 21_ VGGRS 26_ CEELT 31_ AVLTP 36_ PQLLG 41_ RRFNF 46_ FIQQK 51_ CGFRK 56_ APRKV 61_ EPRRS 66_ DPGTS 71_ GEAYK 76_ RSALI 81_ PPVEE 86_ TVFYP 91_ SPYPI 96_ RSLIK 101_ PLFFT 106_ VGFTG 111_ CAFGS 116_ AAIWQ 121_ YESLK 126_ SRVQS 131_ YFDGI 136_ KADWL 141_ DSIRP 146_ QKEGD 151_ FRKEI 156_ NKWWN 161_ NLSDG 166_ QRTVT 171_ GIIAA 176_ NVLVF 181_ CLWRV 186_ PSLQR 191_ TMIRY 196_ FTSNP 201_ ASKVL 206_ CSPML 211_ LSTFS 216_ HFSLF 221_ HMAAN 226_ MYVLW 231_ SFSSS 236_ IVNIL 241_ GQEQF 246_ MAVYL 251_ SAGVI 256_ SNFVS 261_ YVGKV 266_ ATGRY 271_ GPSLG 276_ ASGAI 281_ MTVLA 286_ AVCTK 291_ IPEGR 296_ LAIIF 301_ LPMFT 306_ FTAGN 311_ ALKAI 316_ IAMDT 321_ AGMIL 326_ GWKFF 331_ DHAAH 336_ LGGAL 341_ FGIWY 346_ VTYGH 351_ ELIWK 356_ NREPL 361_ VKIWH 366_ EIRTN 371_GPKKG
1: Required for the control of apoptosis during postnatal growth. Essential for proteolytic processing of an antiapoptotic form of OPA1 which prevents the release of mitochondrial cytochrome c in response to intrinsic apoptotic signals (By similarity). Required for the maturation of PINK1 into its 52kDa mature form after its cleavage by mitochondrial-processing peptidase (MPP) (PubMed:22354088). Promotes cleavage of serine/threonine-protein phosphatase PGAM5 in damaged mitochondria in response to loss of mitochondrial membrane potential (PubMed:22915595). Mediates differential cleavage of PINK1 and PGAM5 depending on the health status of mitochondria, disassociating from PINK1 and associating with PGAM5 in response to mitochondrial membrane potential loss (PubMed:22915595). Required for processing of CLPB into a form with higher protein disaggregase activity by removing an autoinhibitory N-terminal peptide (PubMed:28288130, PubMed:32573439). Promotes processing of DIABLO/SMAC in the mitochondrion which is required for DIABLO apoptotic activity (PubMed:28288130). Also required for cleavage of STARD7 and TTC19 (PubMed:28288130). Promotes changes in mitochondria morphology regulated by phosphorylation of P-beta domain (PubMed:14732705, PubMed:17116872)