Metalloendopeptidase OMA1, mitochondrial (EC 3.4.24.-) (Metalloprotease-related protein 1) (MPRP-1) (Overlapping with the m-AAA protease 1 homolog)
1_MSFIC 6_ GLQSA 11_ ARNHV 16_ FFRFN 21_ SLSNW 26_ RKCNT 31_ LASTS 36_ RGCHQ 41_ VQVNH 46_ IVNKY 51_ QGLGV 56_ NQCDR 61_ WSFLP 66_ GNFHF 71_ YSTFN 76_ NKRTG 81_ GLSST 86_ KSKEI 91_ WRITS 96_ KCTVW 101_ NDAFS 106_ RQLLI 111_ KEVTA 116_ VPSLS 121_ VLHPL 126_ SPASI 131_ RAIRN 136_ FHTSP 141_ RFQAA 146_ PVPLL 151_ LMILK 156_ PVQKL 161_ FAIIV 166_ GRGIR 171_ KWWQA 176_ LPPNK 181_ KEVVK 186_ ENIRK 191_ NKWKL 196_ FLGLS 201_ SFGLL 206_ FVVFY 211_ FTHLE 216_ VSPIT 221_ GRSKL 226_ LLLGK 231_ EQFRL 236_ LSELE 241_ YEAWM 246_ EEFKN 251_ DMLTE 256_ KDARY 261_ LAVKE 266_ VLCHL 271_ IECNK 276_ DVPGI 281_ SQINW 286_ VIHVV 291_ DSPII 296_ NAFVL 301_ PNGQM 306_ FVFTG 311_ FLNSV 316_ TDIHQ 321_ LSFLL 326_ GHEIA 331_ HAVLG 336_ HAAEK 341_ AGMVH 346_ LLDFL 351_ GMIFL 356_ TMIWA 361_ ICPRD 366_ SLALL 371_ CQWIQ 376_ SKLQE 381_ YMFNR 386_ PYSRK 391_ LEAEA 396_ DKIGL 401_ LLAAK 406_ ACADI 411_ RASSV 416_ FWQQM 421_ EFVDS 426_ LHGQP 431_ KMPEW 436_ LSTHP 441_ SHGNR 446_ VEYLD 451_ RLIPQ 456_ ALKIR 461_ EMCNC 466_ PPLSN 471_ PDPRL 476_ LFKLS 481_ TKHFL 486_ EESEK 491_ EDLNI 496_ TKKQK 501_ MDTLP 506_ IQKQE 511_ QIPLT 516_YIVEK
1: Metalloprotease that is part of the quality control system in the inner membrane of mitochondria (PubMed:20038677, PubMed:25605331, PubMed:32132706, PubMed:32132707). Activated in response to various mitochondrial stress, leading to the proteolytic cleavage of target proteins, such as OPA1, UQCC3 and DELE1 (PubMed:20038677, PubMed:25275009, PubMed:32132706, PubMed:32132707). Involved in the fusion of the mitochondrial inner membranes by mediating cleavage of OPA1 at S1 position, generating the soluble OPA1 (S-OPA1), which cooperates with the membrane form (L-OPA1) to coordinate the fusion of mitochondrial inner membranes (PubMed:31922487). Following stress conditions that induce loss of mitochondrial membrane potential, mediates cleavage of OPA1, leading to excess production of soluble OPA1 (S-OPA1) and negative regulation of mitochondrial fusion (PubMed:20038677, PubMed:25275009). Involved in mitochondrial safeguard in response to transient mitochondrial membrane depolarization (flickering) by catalyzing cleavage of OPA1, leading to excess production of S-OPA1, preventing mitochondrial hyperfusion (By similarity). Also acts as a regulator of apoptosis: upon BAK and BAX aggregation, mediates cleavage of OPA1, leading to the remodeling of mitochondrial cristae and allowing the release of cytochrome c from mitochondrial cristae (PubMed:25275009). In depolarized mitochondria, may also act as a backup protease for PINK1 by mediating PINK1 cleavage and promoting its subsequent degradation by the proteasome (PubMed:30733118). May also cleave UQCC3 in response to mitochondrial depolarization (PubMed:25605331). Also acts as an activator of the integrated stress response (ISR): in response to mitochondrial stress, mediates cleavage of DELE1 to generate the processed form of DELE1 (S-DELE1), which translocates to the cytosol and activates EIF2AK1/HRI to trigger the ISR (PubMed:32132706, PubMed:32132707). Its role in mitochondrial quality control is essential for regulating lipid metabolism as well as to maintain body temperature and energy expenditure under cold-stress conditions (By similarity). Binds cardiolipin, possibly regulating its protein turnover (By similarity). Required for the stability of the respiratory supercomplexes (By similarity)