ATPase family AAA domain-containing protein 3A
1_MSWLF 6_ GINKG 11_ PKGEG 16_ AGPPP 21_ PLPPA 26_ QPGAE 31_ GGGDR 36_ GLGDR 41_ PAPKD 46_ KWSNF 51_ DPTGL 56_ ERAAK 61_ AAREL 66_ EHSRY 71_ AKDAL 76_ NLAQM 81_ QEQTL 86_ QLEQQ 91_ SKLKM 96_ RLEAL 101_ SLLHT 106_ LVWAW 111_ SLCRA 116_ GAVQT 121_ QERLS 126_ GSASP 131_ EQVPA 136_ GECCA 141_ LQEYE 146_ AAVEQ 151_ LKSEQ 156_ IRAQA 161_ EERRK 166_ TLSEE 171_ TRQHQ 176_ ARAQY 181_ QDKLA 186_ RQRYE 191_ DQLKQ 196_ QQLLN 201_ EENLR 206_ KQEES 211_ VQKQE 216_ AMRRA 221_ TVERE 226_ MELRH 231_ KNEML 236_ RVEAE 241_ ARARA 246_ KAERE 251_ NADII 256_ REQIR 261_ LKAAE 266_ HRQTV 271_ LESIR 276_ TAGTL 281_ FGEGF 286_ RAFVT 291_ DWDKV 296_ TATVA 301_ GLTLL 306_ AVGVY 311_ SAKNA 316_ TLVAG 321_ RFIEA 326_ RLGKP 331_ SLVRE 336_ TSRIT 341_ VLEAL 346_ RHPIQ 351_ VSRRL 356_ LSRPQ 361_ DALEG 366_ VVLSP 371_ SLEAR 376_ VRDIA 381_ IATRN 386_ TKKNR 391_ SLYRN 396_ ILMYG 401_ PPGTG 406_ KTLFA 411_ KKLAL 416_ HSGMD 421_ YAIMT 426_ GGDVA 431_ PMGRE 436_ GVTAM 441_ HKLFD 446_ WANTS 451_ RRGLL 456_ LFVDE 461_ ADAFL 466_ RKRAT 471_ EKISE 476_ DLRAT 481_ LNAFL 486_ YRTGQ 491_ HSNKF 496_ MLVLA 501_ SNQPE 506_ QFDWA 511_ INDRI 516_ NEMVH 521_ FDLPG 526_ QEERE 531_ RLVRM 536_ YFDKY 541_ VLKPA 546_ TEGKQ 551_ RLKLA 556_ QFDYG 561_ RKCSE 566_ VARLT 571_ EGMSG 576_ REIAQ 581_ LAVSW 586_ QATAY 591_ ASEDG 596_ VLTEA 601_ MMDTR 606_ VQDAV 611_ QQHQQ 616_ KMCWL 621_ KAEGP 626_GRGDE
1: Essential for mitochondrial network organization, mitochondrial metabolism and cell growth at organism and cellular level (PubMed:17210950, PubMed:20154147, PubMed:22453275, PubMed:31522117, PubMed:37832546, PubMed:39116259). May play an important role in mitochondrial protein synthesis (PubMed:22453275). May also participate in mitochondrial DNA replication (PubMed:17210950). May bind to mitochondrial DNA D-loops and contribute to nucleoid stability (PubMed:17210950). Required for enhanced channeling of cholesterol for hormone-dependent steroidogenesis (PubMed:22453275). Involved in mitochondrial-mediated antiviral innate immunity (PubMed:31522117). Required to protect mitochondria from the PERK-mediated unfolded protein response: specifically inhibits the activity of EIF2AK3/PERK at mitochondria-endoplasmic reticulum contact sites, thereby providing a safe haven for mitochondrial protein translation during endoplasmic reticulum stress (PubMed:39116259). Ability to inhibit EIF2AK3/PERK is independent of its ATPase activity (PubMed:39116259). Also involved in the mitochondrial DNA damage response by promoting signaling between damaged genomes and the mitochondrial membrane, leading to activation of the integrated stress response (ISR) (PubMed:37832546)