{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T15:19:52Z","timestamp":1793200792077,"version":"4.5.0"},"reference-count":63,"publisher":"Elsevier BV","issue":"8","license":[{"start":{"date-parts":[[2020,8,1]],"date-time":"2020-08-01T00:00:00Z","timestamp":1596240000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,8,1]],"date-time":"2020-08-01T00:00:00Z","timestamp":1596240000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2020,4,20]],"date-time":"2020-04-20T00:00:00Z","timestamp":1587340800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003246","name":"Nederlandse Organisatie voor Wetenschappelijk Onderzoek","doi-asserted-by":"publisher","award":["CSBR09\/013V"],"award-info":[{"award-number":["CSBR09\/013V"]}],"id":[{"id":"10.13039\/501100003246","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003246","name":"Nederlandse Organisatie voor Wetenschappelijk Onderzoek","doi-asserted-by":"publisher","award":["09\/013V"],"award-info":[{"award-number":["09\/013V"]}],"id":[{"id":"10.13039\/501100003246","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004281","name":"Narodowe Centrum Nauki","doi-asserted-by":"publisher","award":["UMO-2014\/15\/B\/NZ1\/00490"],"award-info":[{"award-number":["UMO-2014\/15\/B\/NZ1\/00490"]}],"id":[{"id":"10.13039\/501100004281","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Biochimica et Biophysica Acta (BBA) - Bioenergetics"],"published-print":{"date-parts":[[2020,8]]},"DOI":"10.1016\/j.bbabio.2020.148213","type":"journal-article","created":{"date-parts":[[2020,4,23]],"date-time":"2020-04-23T02:24:30Z","timestamp":1587608670000},"page":"148213","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":47,"title":["NDUFS4 deletion triggers loss of NDUFA12 in Ndufs4 mice and Leigh syndrome patients: A stabilizing role for NDUFAF2"],"prefix":"10.1016","volume":"1861","author":[{"given":"Merel J.W.","family":"Adjobo-Hermans","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ria","family":"de Haas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter H.G.M.","family":"Willems","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aleksandra","family":"Wojtala","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sjenet E.","family":"van Emst-de Vries","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jori A.","family":"Wagenaars","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariel","family":"van den Brand","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard J.","family":"Rodenburg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan A.M.","family":"Smeitink","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leo G.","family":"Nijtmans","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leonid A.","family":"Sazanov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariusz R.","family":"Wieckowski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Werner J.H.","family":"Koopman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.bbabio.2020.148213_bb0005","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1038\/35072063","article-title":"The genetics and pathology of oxidative phosphorylation","volume":"2","author":"Smeitink","year":"2001","journal-title":"Nat. Rev. Genet."},{"key":"10.1016\/j.bbabio.2020.148213_bb0010","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1038\/191144a0","article-title":"Coupling of phosphorylation to electron and hydrogen transfer by a chemi-osmotic type of mechanism","volume":"191","author":"Mitchell","year":"1961","journal-title":"Nature"},{"key":"10.1016\/j.bbabio.2020.148213_bb0015","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1146\/annurev.biochem.75.103004.142539","article-title":"Energy converting NADH:ubiquinone oxidoreductase (complex I)","volume":"75","author":"Brandt","year":"2006","journal-title":"Annu. Rev. Biochem."},{"key":"10.1016\/j.bbabio.2020.148213_bb0020","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1038\/nature19794","article-title":"Atomic structure of the entire mammalian mitochondrial complex I","volume":"538","author":"Fiedorczuk","year":"2016","journal-title":"Nature"},{"key":"10.1016\/j.bbabio.2020.148213_bb0025","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1038\/nature19095","article-title":"Structure of mammalian respiratory complex I","volume":"536","author":"Zhu","year":"2016","journal-title":"Nature"},{"key":"10.1016\/j.bbabio.2020.148213_bb0030","doi-asserted-by":"crossref","first-page":"980","DOI":"10.1016\/j.bbabio.2016.03.031","article-title":"Unraveling the complexity of mitochondrial complex I assembly: a dynamic process","volume":"1857","author":"S\u00e1nchez-Caballero","year":"2016","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbabio.2020.148213_bb0035","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1038\/nature19754","article-title":"Accessory subunits are integral for assembly and function of human mitochondrial complex I","volume":"538","author":"Stroud","year":"2016","journal-title":"Nature"},{"key":"10.1016\/j.bbabio.2020.148213_bb0040","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1016\/j.cmet.2016.09.002","article-title":"The assembly pathway of mitochondrial respiratory chain complex I","volume":"25","author":"Guerrero-Castillo","year":"2016","journal-title":"Cell Metab."},{"key":"10.1016\/j.bbabio.2020.148213_bb0045","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1038\/nature11871","article-title":"Crystal structure of the entire respiratory complex I","volume":"494","author":"Baradaran","year":"2013","journal-title":"Nature"},{"key":"10.1016\/j.bbabio.2020.148213_bb0050","doi-asserted-by":"crossref","first-page":"448","DOI":"10.1126\/science.1191046","article-title":"Functional modules and structural basis of conformational coupling in mitochondrial complex I","volume":"329","author":"Hunte","year":"2010","journal-title":"Science"},{"key":"10.1016\/j.bbabio.2020.148213_bb0055","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1038\/nature13686","article-title":"Architecture of mammalian respiratory complex I","volume":"515","author":"Vinothkumar","year":"2014","journal-title":"Nature"},{"key":"10.1016\/j.bbabio.2020.148213_bb0060","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1126\/science.1259859","article-title":"Structural biology. Mechanistic insight from the crystal structure of mitochondrial complex I","volume":"347","author":"Zickermann","year":"2015","journal-title":"Science"},{"key":"10.1016\/j.bbabio.2020.148213_bb0065","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1038\/nature19359","article-title":"The architecture of the mammalian respirasome","volume":"537","author":"Gu","year":"2016","journal-title":"Nature"},{"key":"10.1016\/j.bbabio.2020.148213_bb0070","doi-asserted-by":"crossref","DOI":"10.1126\/sciadv.aax9484","article-title":"High-resolution cryo-EM structures of respiratory complex I: mechanism, assembly, and disease","volume":"5","author":"Parey","year":"2019","journal-title":"Sci. Adv."},{"key":"10.1016\/j.bbabio.2020.148213_bb0075","doi-asserted-by":"crossref","first-page":"991","DOI":"10.1016\/j.bbabio.2016.01.013","article-title":"Complex I function in mitochondrial supercomplexes","volume":"1857","author":"Lenaz","year":"2016","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbabio.2020.148213_bb0080","doi-asserted-by":"crossref","first-page":"644","DOI":"10.1038\/nature19774","article-title":"The architecture of respiratory supercomplexes","volume":"537","author":"Letts","year":"2016","journal-title":"Nature"},{"key":"10.1016\/j.bbabio.2020.148213_bb0085","doi-asserted-by":"crossref","first-page":"1598","DOI":"10.1016\/j.cell.2016.11.012","article-title":"Structure of mammalian respiratory supercomplex I1III2IV1","volume":"167","author":"Wu","year":"2016","journal-title":"Cell"},{"key":"10.1016\/j.bbabio.2020.148213_bb0090","doi-asserted-by":"crossref","first-page":"1247","DOI":"10.1016\/j.cell.2017.07.050","article-title":"Architecture of human mitochondrial respiratory megacomplex I2III2IV2","volume":"170","author":"Guo","year":"2017","journal-title":"Cell"},{"key":"10.1016\/j.bbabio.2020.148213_bb0095","doi-asserted-by":"crossref","first-page":"800","DOI":"10.1038\/nsmb.3460","article-title":"Clarifying the supercomplex: the higher-order organization of the mitochondrial electron transport chain","volume":"24","author":"Letts","year":"2017","journal-title":"Nat. Struct. Mol. Biol."},{"key":"10.1016\/j.bbabio.2020.148213_bb0100","doi-asserted-by":"crossref","first-page":"16080","DOI":"10.1038\/nrdp.2016.80","article-title":"Mitochondrial diseases","volume":"2","author":"Gorman","year":"2016","journal-title":"Nat. Rev. Dis. Primers"},{"key":"10.1016\/j.bbabio.2020.148213_bb0105","doi-asserted-by":"crossref","first-page":"938","DOI":"10.1016\/j.bbabio.2016.02.012","article-title":"Mitochondrial complex I-linked disease","volume":"1857","author":"Rodenburg","year":"2016","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbabio.2020.148213_bb0110","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1136\/jnnp-2012-304426","article-title":"A guide to diagnosis and treatment of Leigh syndrome","volume":"85","author":"Baertling","year":"2014","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"10.1016\/j.bbabio.2020.148213_bb0115","doi-asserted-by":"crossref","first-page":"44161","DOI":"10.1074\/jbc.M307615200","article-title":"Pathological mutations of the human NDUFS4 gene of the 18-kDa (AQDQ) subunit of complex I affect the expression of the protein and the assembly and function of the complex","volume":"278","author":"Scacco","year":"2003","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbabio.2020.148213_bb0120","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1093\/hmg\/ddh071","article-title":"Differences in assembly or stability of complex I and other mitochondrial OXPHOS complexes in inherited complex I deficiency","volume":"13","author":"Ugalde","year":"2004","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbabio.2020.148213_bb0125","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1002\/ddrr.107","article-title":"Complex I disorders: causes, mechanisms and development of treatment strategies at the cellular level","volume":"16","author":"Valsecchi","year":"2010","journal-title":"Development. Disab. Res. Rev."},{"key":"10.1016\/j.bbabio.2020.148213_bb0130","doi-asserted-by":"crossref","first-page":"568","DOI":"10.1016\/j.febslet.2011.09.019","article-title":"Respiratory chain complex I, a main regulatory target of the cAMP\/PKA pathway is defective in different human diseases","volume":"586","author":"Papa","year":"2012","journal-title":"FEBS Lett."},{"key":"10.1016\/j.bbabio.2020.148213_bb0135","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1016\/j.bbabio.2016.11.010","article-title":"Accessory subunit NUYM (NDUFS4) is required for stability of the electron input module and activity of mitochondrial complex I","volume":"1858","author":"Kahlh\u00f6fer","year":"2017","journal-title":"Biochim. Biophys. Acta Bioenerg."},{"key":"10.1016\/j.bbabio.2020.148213_bb0140","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1016\/j.mito.2016.04.001","article-title":"Ndufs4 related Leigh syndrome: a case report and review of the literature","volume":"28","author":"Ortigoza-Escobar","year":"2016","journal-title":"Mitochondrion"},{"key":"10.1016\/j.bbabio.2020.148213_bb0145","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.mito.2011.06.011","article-title":"Mini-review: pharmacological targeting of complex I deficiency: the cellular level and beyond","volume":"12","author":"Roestenberg","year":"2012","journal-title":"Mitochondrion"},{"key":"10.1016\/j.bbabio.2020.148213_bb0150","doi-asserted-by":"crossref","first-page":"11733","DOI":"10.1038\/s41598-017-09417-5","article-title":"Therapeutic effects of the mitochondrial ROS-redox modulator KH176 in a mammalian model of Leigh Disease","volume":"7","author":"de Haas","year":"2017","journal-title":"Sci. Rep."},{"key":"10.1016\/j.bbabio.2020.148213_bb0155","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1016\/j.cmet.2008.02.004","article-title":"Mice with mitochondrial complex I deficiency develop a fatal encephalomyopathy","volume":"7","author":"Kruse","year":"2008","journal-title":"Cell Metab."},{"key":"10.1016\/j.bbabio.2020.148213_bb0160","doi-asserted-by":"crossref","first-page":"15136","DOI":"10.1073\/pnas.0807581105","article-title":"Mitochondrial complex I inhibition is not required for dopaminergic neuron death induced by rotenone, MPP+, or paraquat","volume":"105","author":"Choi","year":"2008","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.bbabio.2020.148213_bb0165","doi-asserted-by":"crossref","first-page":"873","DOI":"10.1083\/jcb.201009132","article-title":"Loss of mitochondrial complex I activity potentiates dopamine neuron death induced by microtubule dysfunction in a Parkinson\u2019s disease model","volume":"192","author":"Choi","year":"2011","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbabio.2020.148213_bb0170","doi-asserted-by":"crossref","first-page":"10996","DOI":"10.1073\/pnas.1006214107","article-title":"Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome","volume":"107","author":"Quintana","year":"2010","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.bbabio.2020.148213_bb0175","doi-asserted-by":"crossref","first-page":"2359","DOI":"10.1172\/JCI62923","article-title":"Fatal breathing dysfunction in a mouse model of Leigh syndrome","volume":"122","author":"Quintana","year":"2012","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.bbabio.2020.148213_bb0180","doi-asserted-by":"crossref","first-page":"1078","DOI":"10.1093\/hmg\/ddr537","article-title":"Altered dopamine metabolism and increased vulnerability to MPTP in mice with partial deficiency of mitochondrial complex I in dopamine neurons","volume":"21","author":"Sterky","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbabio.2020.148213_bb0185","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1016\/j.cmet.2013.07.002","article-title":"Mitochondrial complex I deficiency increases protein acetylation and accelerates heart failure","volume":"18","author":"Karamanlidis","year":"2013","journal-title":"Cell Metab."},{"key":"10.1016\/j.bbabio.2020.148213_bb0190","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1002\/iub.1127","article-title":"Examining the validity of in vitro and in vivo models for Ndufs4 mutations in respiratory complex I","volume":"65","author":"Breuer","year":"2013","journal-title":"IUBMB Life"},{"key":"10.1016\/j.bbabio.2020.148213_bb0195","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1016\/j.bbr.2015.09.006","article-title":"Gait analysis in a mouse model resembling Leigh disease","volume":"296","author":"de Haas","year":"2016","journal-title":"Behav. Brain Res."},{"key":"10.1016\/j.bbabio.2020.148213_bb0200","doi-asserted-by":"crossref","first-page":"1925","DOI":"10.1016\/j.bbabio.2012.03.006","article-title":"Metabolic consequences of Ndufs4 gene deletion in immortalized mouse embryonic fibroblasts","volume":"1817","author":"Valsecchi","year":"2012","journal-title":"Biochim. Biophys. Acta Bioenerg."},{"key":"10.1016\/j.bbabio.2020.148213_bb0205","doi-asserted-by":"crossref","first-page":"C881","DOI":"10.1152\/ajpcell.00104.2005","article-title":"Mitochondrial network complexity and pathological decrease in complex I activity are tightly correlated in isolated human complex I deficiency","volume":"289","author":"Koopman","year":"2005","journal-title":"Am. J. Phys. Cell Physiol."},{"key":"10.1016\/j.bbabio.2020.148213_bb0210","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1007\/s00109-009-0452-5","article-title":"The antioxidant Trolox restores mitochondrial membrane potential and Ca2+-stimulated ATP production in human complex I deficiency","volume":"87","author":"Distelmaier","year":"2009","journal-title":"J. Mol. Med."},{"key":"10.1016\/j.bbabio.2020.148213_bb0215","doi-asserted-by":"crossref","first-page":"833","DOI":"10.1093\/brain\/awp058","article-title":"Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease","volume":"132","author":"Distelmaier","year":"2009","journal-title":"Brain"},{"key":"10.1016\/j.bbabio.2020.148213_bb0220","doi-asserted-by":"crossref","first-page":"1773","DOI":"10.1016\/j.biocel.2009.01.012","article-title":"Mitochondrial dynamics in human NADH:oxidoreductase deficiency","volume":"41","author":"Willems","year":"2009","journal-title":"Int. J. Biochem. Cell Biol."},{"key":"10.1016\/j.bbabio.2020.148213_bb0225","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1111\/j.2517-6161.1995.tb02031.x","article-title":"Controlling the false discovery rate: a practical and powerful approach to multiple testing","volume":"57","author":"Benjamini","year":"1995","journal-title":"J. Royal Stat. Soc. B"},{"key":"10.1016\/j.bbabio.2020.148213_bb0230","doi-asserted-by":"crossref","first-page":"236","DOI":"10.1080\/01621459.1963.10500845","article-title":"Hierarchical grouping to optimize an objective function","volume":"58","author":"Ward","year":"1963","journal-title":"J. Am. Stat. Assoc."},{"key":"10.1016\/j.bbabio.2020.148213_bb0235","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1038\/nprot.2015.053","article-title":"The Phyre2 web portal for protein modeling, prediction and analysis","volume":"10","author":"Kelley","year":"2015","journal-title":"Nat. Protoc."},{"key":"10.1016\/j.bbabio.2020.148213_bb0240","doi-asserted-by":"crossref","first-page":"1685","DOI":"10.1074\/jbc.C112.436766","article-title":"Gene knockout using transcription activator-like effector nucleases (TALENs) reveals that human NDUFA9 protein is essential for stabilizing the junction between membrane and matrix arms of complex I","volume":"288","author":"Stroud","year":"2013","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbabio.2020.148213_bb0245","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1042\/bj20021594","article-title":"Mitochondrial threshold effects","volume":"370","author":"Rossignol","year":"2003","journal-title":"Biochem. J."},{"issue":"3","key":"10.1016\/j.bbabio.2020.148213_bb0250","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1016\/j.bbabio.2016.12.001","article-title":"Subunit NDUFV3 is present in two distinct isoforms in mammalian complex I","volume":"1858","author":"Bridges","year":"2017","journal-title":"Biochim. Biophys. Acta Bioenerg."},{"key":"10.1016\/j.bbabio.2020.148213_bb0255","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1002\/1873-3468.12527","article-title":"A novel isoform of the human mitochondrial complex I subunit NDUFV3","volume":"591","author":"Dibley","year":"2017","journal-title":"FEBS Lett."},{"key":"10.1016\/j.bbabio.2020.148213_bb0260","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1016\/j.bbabio.2016.12.004","article-title":"Identification and evolutionary analysis of tissue-specific isoforms of mitochondrial complex I subunit NDUFV3","volume":"858","author":"Guerrero-Castillo","year":"2017","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbabio.2020.148213_bb0265","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1074\/mcp.RA119.001784","article-title":"The mitochondrial acyl-carrier protein interaction network highlights important roles for LYRM family members in complex I and mitoribosome assembly","volume":"19","author":"Dibley","year":"2020","journal-title":"Mol. Cell. Proteomics"},{"key":"10.1016\/j.bbabio.2020.148213_bb0270","doi-asserted-by":"crossref","first-page":"765","DOI":"10.1016\/j.cmet.2017.03.009","article-title":"The enigma of the respiratory chain supercomplex","volume":"25","author":"Milenkovic","year":"2017","journal-title":"Cell Metab."},{"key":"10.1016\/j.bbabio.2020.148213_bb0275","doi-asserted-by":"crossref","first-page":"2784","DOI":"10.1172\/JCI26020","article-title":"A molecular chaperone for mitochondrial complex I assembly is mutated in a progressive encephalopathy","volume":"115","author":"Ogilvie","year":"2005","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.bbabio.2020.148213_bb0280","doi-asserted-by":"crossref","first-page":"176","DOI":"10.1016\/j.ymgme.2007.02.007","article-title":"Investigation of the complex I assembly chaperones B17.2L and NDUFAF1 in a cohort of CI deficient patients","volume":"91","author":"Vogel","year":"2007","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.bbabio.2020.148213_bb0285","doi-asserted-by":"crossref","first-page":"4228","DOI":"10.1128\/MCB.00074-07","article-title":"Analysis of the assembly profiles for mitochondrial- and nuclear-DNA-encoded subunits into complex I","volume":"27","author":"Lazarou","year":"2007","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.bbabio.2020.148213_bb0290","doi-asserted-by":"crossref","first-page":"5685","DOI":"10.1073\/pnas.1424353112","article-title":"Accessory NUMM (NDUFS6) subunit harbors a Zn-binding site and is essential for biogenesis of mitochondrial complex I","volume":"112","author":"Kmita","year":"2015","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.bbabio.2020.148213_bb0295","doi-asserted-by":"crossref","first-page":"2623","DOI":"10.1128\/MCB.01476-12","article-title":"Novel insights into the role of Neurospora crassa NDUFAF2, an evolutionarily conserved mitochondrial complex I assembly factor","volume":"33","author":"Pereira","year":"2013","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.bbabio.2020.148213_bb0300","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1093\/hmg\/ddr446","article-title":"Mitochondrial complex III stabilizes complex I in the absence of NDUFS4 to provide partial activity","volume":"21","author":"Calvaruso","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbabio.2020.148213_bb0305","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.nbd.2013.05.007","article-title":"The mitochondrial disease associated protein Ndufaf2 is dispensable for complex-1 assembly but critical for the regulation of oxidative stress","volume":"58","author":"Schlehe","year":"2013","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.bbabio.2020.148213_bb0310","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1038\/nrm.2017.138","article-title":"Understanding the diversity of membrane lipid composition","volume":"19","author":"Harayama","year":"2018","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.bbabio.2020.148213_bb0315","doi-asserted-by":"crossref","first-page":"7517","DOI":"10.1074\/jbc.R117.001655","article-title":"Lipid-mediated signals that regulate mitochondrial biology","volume":"293","author":"Nielson","year":"2018","journal-title":"J. Biol. Chem."}],"container-title":["Biochimica et Biophysica Acta (BBA) - Bioenergetics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0005272820300633?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0005272820300633?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T11:56:05Z","timestamp":1777377365000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0005272820300633"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,8]]},"references-count":63,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2020,8]]}},"alternative-id":["S0005272820300633"],"URL":"https:\/\/doi.org\/10.1016\/j.bbabio.2020.148213","relation":{},"ISSN":["0005-2728"],"issn-type":[{"value":"0005-2728","type":"print"}],"subject":[],"published":{"date-parts":[[2020,8]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"NDUFS4 deletion triggers loss of NDUFA12 in Ndufs4\u2212\/\u2212 mice and Leigh syndrome patients: A stabilizing role for NDUFAF2","name":"articletitle","label":"Article Title"},{"value":"Biochimica et Biophysica Acta (BBA) - Bioenergetics","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.bbabio.2020.148213","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2020 The Author(s). Published by Elsevier B.V.","name":"copyright","label":"Copyright"}],"article-number":"148213"}}