Quality control of the mitochondrion

نویسندگان

چکیده

Mitochondria are essential organelles that execute and coordinate various metabolic processes in the cell. Mitochondrial dysfunction severely affects cell fitness contributes to disease. Proper organellar function depends on biogenesis maintenance of mitochondria its >1,000 proteins. As a result, has evolved mechanisms protein quality control, such as turnover proteins via mitochondria-associated degradation, ubiquitin-proteasome system, mitoproteases, well elimination through mitophagy. Specific control engaged depending upon nature severity mitochondrial dysfunction, which can also feed back elicit transcriptional or proteomic remodeling by Here, we will discuss current understanding how these different integrated overlap maintain they may be relevant for cellular organismal health. specialized act hubs signaling platforms, involved an array ATP production oxidative phosphorylation (OXPHOS), fatty acid oxidation, calcium buffering, phospholipid synthesis, reactive oxygen species generation maintenance, iron-sulfur cluster biosynthesis, innate immune (Spinelli Haigis, 2018Spinelli J.B. Haigis M.C. The multifaceted contributions metabolism.Nat. Cell Biol. 2018; 20: 745-754Crossref PubMed Scopus (187) Google Scholar). In contrast 13 DNA (mtDNA)-encoded proteins, more than 1,200 nuclear-encoded (NUMPs) first synthesized cytosol then targeted submitochondrial compartments: outer membrane (OMM), intermembrane space (IMS), inner (IMM), matrix (Wiedemann Pfanner, 2017Wiedemann N. Pfanner machineries import assembly.Annu. Rev. Biochem. 2017; 86: 685-714Crossref (254) It is imperative NUMPs properly processed, folded, localized Scholar), interference at any point along this chain events detrimental Accordingly, overlapping interconnected safeguard have mitigate damage occur result external insults nutrient deprivation, hypoxia, OXPHOS uncoupling, consequence aberrant proteostasis. These include checkpoints, acting prior import, mitochondrial-associated degradation (MAD) other linked ubiquitin (Ub)-proteasome system (UPS). Inside mitochondria, proteases (mitoproteases) regulate imported mtDNA-encoded gene products. Finally, checkpoints involve segregation, removal, fragments mitochondrial-derived vesicles (MDVs) entire (mitophagy) nuclear responses (unfolded response (UPRmt)) signals. review systems (Figure 1) molecular connected coordinated. proteome estimated contain humans (Rath et al., 2021Rath S. Sharma R. Gupta Ast T. Chan C. Durham T.J. Goodman R.P. Grabarek Z. Haas M.E. Hung W.H.W. al.MitoCarta3.0: updated now with sub-organelle localization pathway annotations.Nucleic Acids Res. 2021; 49: D1541-D1547Crossref (15) Scholar) around 900–1,000 yeast (Morgenstern 2017Morgenstern M. Stiller S.B. Lübbert P. Peikert C.D. Dannenmaier Drepper F. Weill U. Höß Feuerstein Gebert al.Definition high-confidence quantitative scale.Cell Rep. 19: 2836-2852Abstract Full Text PDF (137) Scholar; Vögtle 2017Vögtle F.N. Burkhart J.M. Gonczarowska-Jorge H. Kücükköse Taskin A.A. Kopczynski D. Ahrends Mossmann Sickmann A. Zahedi Meisinger Landscape distribution.Nat. Commun. 8: 290Crossref (53) Virtually all their targeting coordinated regulated manner (Bykov 2020Bykov Y.S. Rapaport Herrmann Schuldiner Cytosolic proteins.Trends Sci. 2020; 45: 650-667Abstract (12) precursor kept unfolded Hsp70 Hsp90 family cytosolic chaperones delivery mitochondria. often possess short N-terminal peptide, composed hydrophobic positively charged amino acids, facilitating interaction negatively residues surface translocase OMM (TOM) complex primary channel However, prematurely fold stall during translocation, seen when potential lost (Weidberg Amon, 2018Weidberg Amon MitoCPR-a surveillance protects stress.Science. 360: eaan4146Crossref (106) leading accumulation aberrantly elsewhere cell, makes them accessible 2). One MAD analogous endoplasmic reticulum (ER) mechanism called ER-associated (ERAD) (Hwang Qi, 2018Hwang J. Qi L. Quality reticulum: crosstalk between ERAD UPR pathways.Trends 43: 593-605Abstract (92) facilitates ubiquitination turnover. During MAD, type II AAA+ ATPase VCP/p97 (Cdc48 yeast) hydrolyzes from facilitate extraction proteasome 2, MAD) (Xu 2011Xu Peng G. Wang Y. Fang Karbowski AAA-ATPase p97 turnover.Mol. Cell. 2011; 22: 291-300Crossref (168) indicating important role homeostasis. Substrates factors morphology (Fzo1 [Cohen 2008Cohen M.M. Leboucher G.P. Livnat-Levanon Glickman M.H. Weissman A.M. Ubiquitin-proteasome-dependent mitofusin, critical regulator fusion.Mol. 2008; 2457-2464Crossref (131) Scholar], Mitofusins Mcl-1 mammals [Tanaka 2010Tanaka Cleland Xu Narendra D.P. Suen D.F. Youle R.J. Proteasome mediate mitophagy mitofusins induced Parkin.J. 2010; 191: 1367-1380Crossref (849) ER-mitochondrial contact sites [Mdm34p] [Wu 2016bWu X. Li Jiang Doa1 targets ubiquitinated substrates degradation.J. 2016; 213: 49-63Crossref (43) [Msp1p, Tomm70p] Scholar]). Cdc48 capable removing stalled nascent peptides ribosomes, thus (Verma 2013Verma Oania R.S. Kolawa N.J. Deshaies Cdc48/p97 promotes polypeptides bound ribosome.eLife. 2013; 2: e00308Crossref (138) Defenouillère 2013Defenouillère Q. Yao Mouaikel Namane Galopier Decourty Doyen Malabat Saveanu Jacquier Fromont-Racine Cdc48-associated 60S particles required clearance translation products.Proc. Natl. Acad. USA. 110: 5046-5051Crossref (139) Brandman 2012Brandman O. Stewart-Ornstein Wong Larson Williams C.C. G.W. Zhou King Shen P.S. Weibezahn al.A ribosome-bound triggers signals stress.Cell. 2012; 151: 1042-1054Abstract recruited stress (Heo 2010Heo J.-M. Taylor E.B. Jones K.T. Dephoure Ring Xie Brodsky J.L. Madeo Gygi S.P. stress-responsive degradation.Mol. 40: 465-480Abstract (209) cofactor necessary association (Wu including IMS (Liao 2020Liao P.C. Wolken D.M.A. Serrano E. Srivastava Pon L.A. Mitochondria-associated beyond membrane.Cell 32: 107902Abstract (0) although it unclear become ubiquitinated. Interestingly, but no tested, protected cells paraquat-induced stress, main player oxidized damaged yeast, several pathways removal accumulate channels OMM, promoting UPS. translocation-associated (MitoTAD) (Mårtensson 2019Mårtensson C.U. Priesnitz Song Ellenrieder Doan K.N. Boos Floerchinger Zufall Oeljeklaus Warscheid B. Becker degradation.Nature. 2019; 569: 679-683Crossref (63) whose IMM depolarization, following treatment protonophore CCCP, TOM complex. Such arrest Ub-like (UBX)-domain-containing 2 (Ubx2) complex, recruitment Ufd1 remove arrested mitoTAD). Ubx2 further interacts adaptor Vms1, itself ribosome-associated chains ribosomes (Izawa 2017Izawa Park S.H. Zhao Hartl F.U. Neupert W. Vms1 links ribosome homeostasis.Cell. 171: 890-903.e18Abstract (66) Verma 2018Verma Reichermeier K.M. Burroughs Reitsma Aravind ANKZF1 peptidyl-tRNA hydrolases release ribosomes.Nature. 557: 446-451Crossref (50) Zurita Rendón 2018Zurita Fredrickson E.K. Howard C.J. Van Vranken Fogarty Tolley N.D. Kalia Osuna B.A. Hill C.P. al.Vms1p factor complex.Nat. 9: 2197Crossref (37) suggesting expands highlights coordination synthesis import. compromised (MitoCPR) involves UPS precursors destined IMS. signal followed “stop-transfer” sequence This bipartite removed proteolytic cleavage, into IMS, thus, preventing translocation (Neupert Herrmann, 2007Neupert Translocation mitochondria.Annu. 2007; 76: 723-749Crossref (999) When inhibited, translocases. leads activation mitoCPR induce expression Cis1 transcription Pdr3, turn recruits Msp1 extract non-imported mitoCPR). believed associate part translocating unfold early folded precursors, if unsuccessful, target proteasomal (Wang 2020Wang Myasnikov Pan Walter Structure AAA reveals mislocalized extraction.eLife. e54031Crossref extracting mistargeted tail-anchored reinserted ER membrane, where extracted sent (Matsumoto 2019Matsumoto Nakatsukasa K. Kakuta Tamura Esaki Endo clears transfer ER.Mol. 191-205.e10Abstract (26) mitoTAD mainly been elucidated under artificial induction whether exist metazoans triggered physiological stimuli not known. central component (mitoRQC) rescues ribosome-stalled together E3 ligase Ltn1 Rqc2, directly bind Some translocated co-translocation formation inaccessible aggregation addition C-terminal alanyl/threonine (CAT-tails) (Shen 2015Shen Qin Parsawar Cox Cheng Lambowitz J.S. al.Protein synthesis. Rqc2p ribosomal subunits mRNA-independent elongation chains.Science. 2015; 347: 75-78Crossref generated Rqc2 ultimately toxic associates dissociate prevent CAT-tailing (Su 2019Su Izawa Thoms Yamashita Berninghausen Inada Beckmann Arb1 RQC homeostasis.Nature. 570: 538-542Crossref (22) allowing transport polypeptide exposed like mito-proteases (see below). Conversely, ubiquitinates lysine chain, mitoRQC). mitoRQC yet described mammals, interesting note loss human homolog ankyrin repeat zinc-finger-domain-containing 1 (ANKZF1), decreased integrity was able rescue Vms1-deficient (Van Haaften-Visser 2017Van D.Y. Harakalova Mocholi Montfrans Elkadri Rieter Fiedler Hasselt P.M. Triffaux E.M.M. Haelst al.Ankyrin zinc-finger domain-containing mutations associated infantile-onset inflammatory bowel disease.J. Chem. 292: 7904-7920Abstract (7) well-established roles global Kuroha 2018Kuroha Zinoviev Hellen C.U.T. Pestova T.V. Release non-ubiquitinated mammalian complexes Ptrh1.Mol. 72: 286-302.e8Abstract (36) Yip 2019Yip M.C.J. Keszei A.F.A. Feng Chu V. Mckenna M.J. Shao Mechanism recycling tRNAs ribosomes.Nat. Struct. Mol. 26: 343-349Crossref (20) Further supporting link, shown translocate H2O2-induced aggregated (MitoCPR, MitoTAD, mitoRQC), modulated face insults. UPRam alleviates proteotoxic caused inhibition (Wrobel 2015Wrobel Topf Bragoszewski Wiese Sztolsztener Varabyova Lirski Chroscicki Mroczek al.Mistargeted activate proteostatic cytosol.Nature. 524: 485-488Crossref (185) UPRam). While cytoplasmic aggregates typically inhibit (Bennett 2005Bennett E.J. Bence N.F. Jayakumar Kopito R.R. Global impairment precedes inclusion body formation.Mol. 2005; 17: 351-365Abstract (391) stimulates activity inhibits order reduce burden reason behind differential influence fully understood. Accumulation much apoptotic overaccumulation (mPOS) Chen, 2015Wang Chen X.J. A network suppressing mitochondria-mediated death.Nature. 481-484Crossref (156) Cox12 Pet191 reported retro-translocated TOM40 (Bragoszewski 2015Bragoszewski Wasilewski Sakowska Gornicka Böttinger Qiu Wiedemann Chacinska Retro-translocation proteins.Proc. 112: 7713-7718Crossref (61) could increase abundance initiate mPOS. Another death mitochondrial-stress-induced translational termination carboxy-terminal (C-terminal) extension (MISTERMINATE), Drosophila cells, activated reduced mRNA ABCE1 eRF1. Reduction eRF1 non-mRNA encoded I subunit NDUFS3, accumulated both 2019Wu Tantray I. Lim Davis Sitron Dong Gispert Auburger al.MISTERMINATE mechanistically proteostasis failure.Mol. 75: 835-848.e8Abstract Indeed, HeLa rotenone CCCP prompted transcripts Proteasomal possibly initiating step MISTERMINATE, K48-polyubiquitinated lines 2018Wu Liu Vartak Stankiewicz Montgomery Lu Ubiquitination NOT4 co-translational PINK1-directed mitophagy.Cell Metab. 28: 130-144.e7Abstract (21) represent cytosol. Even though many only so far, findings steppingstones future investigations counterparts. allows organelle respond rapidly Ub small 76 acids conjugated (K) wide range process Ub-activating enzymes (E1), Ub-conjugating (E2), ligases (E3), forming polyUb (Deol 2019Deol K.K. Lorenz Strieter E.R. Enzymatic logic assembly.Front. Physiol. 10: 835Crossref conjugated, alter binding Ub-binding domains. functions signal, since tagged K29- K48-linked proteasome, while K63-linked autophagy (discussed below), transduction, endocytosis (Kwon Ciechanover, 2017Kwon Y.T. Ciechanover code autophagy.Trends 42: 873-886Abstract (223) Proteins scrutiny MARCH5 (also known MITOL) misfolded (Yonashiro 2009Yonashiro Sugiura Miyachi Fukuda Matsushita Inatome Ogata Suzuki Dohmae Yanagi MITOL mutant SOD1 attenuates SOD1-induced generation.Mol. 2009; 4524-4530Crossref (79) 2011Sugiura Yonashiro Nagashima controls toxicity polyglutamine-expanded protein.Mitochondrion. 11: 139-146Crossref Figure 1). Additionally, recently mouse neurons region, thereby subsequent (Phu 2020Phu Rose C.M. Tea Wall C.E. Verschueren Cheung T.K. Kirkpatrick D.S. Bingol Dynamic regulation system.Mol. 77: 1107-1123.e10Abstract (16) counter-regulated deubiquitinase USP30, depletion USP30 led generating mito

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ژورنال

عنوان ژورنال: Developmental Cell

سال: 2021

ISSN: ['1878-1551', '1534-5807']

DOI: https://doi.org/10.1016/j.devcel.2021.02.009