000165192 001__ 165192
000165192 005__ 20251219174251.0
000165192 0247_ $$2doi$$a10.15252/embj.2019104105
000165192 0248_ $$2sideral$$a146777
000165192 037__ $$aART-2020-146777
000165192 041__ $$aeng
000165192 100__ $$aStephan, Till
000165192 245__ $$aMICOS assembly controls mitochondrial inner membrane remodeling and crista junction redistribution to mediate cristae formation
000165192 260__ $$c2020
000165192 5060_ $$aAccess copy available to the general public$$fUnrestricted
000165192 5203_ $$aMitochondrial function is critically dependent on the folding of the mitochondrial inner membrane into cristae; indeed, numerous human diseases are associated with aberrant crista morphologies. With the MICOS complex, OPA1 and the F1Fo‐ATP synthase, key players of cristae biogenesis have been identified, yet their interplay is poorly understood. Harnessing super‐resolution light and 3D electron microscopy, we dissect the roles of these proteins in the formation of cristae in human mitochondria. We individually disrupted the genes of all seven MICOS subunits in human cells and re‐expressed Mic10 or Mic60 in the respective knockout cell line. We demonstrate that assembly of the MICOS complex triggers remodeling of pre‐existing unstructured cristae and de novo formation of crista junctions (CJs) on existing cristae. We show that the Mic60‐subcomplex is sufficient for CJ formation, whereas the Mic10‐subcomplex controls lamellar cristae biogenesis. OPA1 stabilizes tubular CJs and, along with the F1Fo‐ATP synthase, fine‐tunes the positioning of the MICOS complex and CJs. We propose a new model of cristae formation, involving the coordinated remodeling of an unstructured crista precursor into multiple lamellar cristae.
000165192 540__ $$9info:eu-repo/semantics/openAccess$$aby$$uhttps://creativecommons.org/licenses/by/4.0/deed.es
000165192 590__ $$a11.598$$b2020
000165192 591__ $$aCELL BIOLOGY$$b21 / 195 = 0.108$$c2020$$dQ1$$eT1
000165192 591__ $$aBIOCHEMISTRY & MOLECULAR BIOLOGY$$b22 / 296 = 0.074$$c2020$$dQ1$$eT1
000165192 592__ $$a7.484$$b2020
000165192 593__ $$aBiochemistry, Genetics and Molecular Biology (miscellaneous)$$c2020$$dQ1
000165192 593__ $$aNeuroscience (miscellaneous)$$c2020$$dQ1
000165192 593__ $$aMolecular Biology$$c2020$$dQ1
000165192 593__ $$aMedicine (miscellaneous)$$c2020$$dQ1
000165192 655_4 $$ainfo:eu-repo/semantics/article$$vinfo:eu-repo/semantics/publishedVersion
000165192 700__ $$aBrüser, Christian
000165192 700__ $$aDeckers, Markus
000165192 700__ $$aSteyer, Anna M.
000165192 700__ $$aBalzarotti, Francisco
000165192 700__ $$aBarbot, Mariam
000165192 700__ $$aBehr, Tiana S.
000165192 700__ $$aHeim, Gudrun
000165192 700__ $$aHübner, Wolfgang
000165192 700__ $$aIlgen, Peter
000165192 700__ $$aLange, Felix
000165192 700__ $$0(orcid)0000-0003-2645-3983$$aPacheu-Grau, David$$uUniversidad de Zaragoza
000165192 700__ $$aPape, Jasmin K.
000165192 700__ $$aStoldt, Stefan
000165192 700__ $$aHuser, Thomas
000165192 700__ $$aHell, Stefan W.
000165192 700__ $$aMöbius, Wiebke
000165192 700__ $$aRehling, Peter
000165192 700__ $$aRiedel, Dietmar
000165192 700__ $$aJakobs, Stefan
000165192 7102_ $$11002$$2060$$aUniversidad de Zaragoza$$bDpto. Bioq.Biolog.Mol. Celular$$cÁrea Bioquímica y Biolog.Mole.
000165192 773__ $$g39, 14 (2020), e104105 [24 pp.]$$pEMBO j.$$tEMBO JOURNAL$$x0261-4189
000165192 8564_ $$s5959848$$uhttps://zaguan.unizar.es/record/165192/files/texto_completo.pdf$$yVersión publicada
000165192 8564_ $$s2696918$$uhttps://zaguan.unizar.es/record/165192/files/texto_completo.jpg?subformat=icon$$xicon$$yVersión publicada
000165192 909CO $$ooai:zaguan.unizar.es:165192$$particulos$$pdriver
000165192 951__ $$a2025-12-19-14:42:46
000165192 980__ $$aARTICLE