Mitochondrial complex I plays an essential role in human respirasome assembly

scientific article published on 16 February 2012

Mitochondrial complex I plays an essential role in human respirasome assembly is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1016/J.CMET.2012.01.015
P932PMC publication ID3318979
P698PubMed publication ID22342700
P5875ResearchGate publication ID221840264

P50authorJoaquín ArenasQ56939581
P2093author name stringCristina Ugalde
Antoni Barrientos
David Moreno-Lastres
Flavia Fontanesi
Inés García-Consuegra
Miguel A Martín
P2860cites workNDUFS6 mutations are a novel cause of lethal neonatal mitochondrial complex I deficiencyQ24304313
Differences in assembly or stability of complex I and other mitochondrial OXPHOS complexes in inherited complex I deficiencyQ24306549
Respiratory complex III is required to maintain complex I in mammalian mitochondriaQ24322645
Mutation of C20orf7 disrupts complex I assembly and causes lethal neonatal mitochondrial diseaseQ24336408
Identification of mitochondrial complex I assembly intermediates by tracing tagged NDUFS3 demonstrates the entry point of mitochondrial subunitsQ24338844
A molecular chaperone for mitochondrial complex I assembly is mutated in a progressive encephalopathyQ24536088
Multiprotein complex containing succinate dehydrogenase confers mitochondrial ATP-sensitive K+ channel activityQ24563970
Supercomplexes in the respiratory chains of yeast and mammalian mitochondriaQ24599994
Analysis of the assembly profiles for mitochondrial- and nuclear-DNA-encoded subunits into complex IQ24683107
Arrangement of electron transport chain components in bovine mitochondrial supercomplex I1III2IV1Q27673724
Assembly of cytochrome-c oxidase in cultured human cellsQ28276105
Cytochrome c oxidase is required for the assembly/stability of respiratory complex I in mouse fibroblastsQ28585444
Human cells lacking mtDNA: repopulation with exogenous mitochondria by complementationQ29614475
Respiratory Chain Complexes in Dynamic Mitochondria Display a Patchy Distribution in Life CellsQ33650944
The ratio of oxidative phosphorylation complexes I-V in bovine heart mitochondria and the composition of respiratory chain supercomplexesQ33953875
Architecture of active mammalian respiratory chain supercomplexes.Q33994333
A structural model of the cytochrome C reductase/oxidase supercomplex from yeast mitochondriaQ34002630
Interaction of complexes I, III, and IV within the bovine respirasome by single particle cryoelectron tomographyQ34028316
Investigation of the complex I assembly chaperones B17.2L and NDUFAF1 in a cohort of CI deficient patientsQ34612081
Impaired complex III assembly associated with BCS1L gene mutations in isolated mitochondrial encephalopathyQ34614921
Blue Native electrophoresis to study mitochondrial and other protein complexesQ34675865
Biogenesis of the yeast cytochrome bc1 complex.Q37172145
Assembly of the oxidative phosphorylation system in humans: what we have learned by studying its defectsQ37213554
Supramolecular organization of ATP synthase and respiratory chain in mitochondrial membranesQ37376950
Synthesis of cytochrome c oxidase subunit 1 is translationally downregulated in the absence of functional F1F0-ATP synthaseQ37394160
Structure and organization of mitochondrial respiratory complexes: a new understanding of an old subjectQ37594110
Assembly factors of human mitochondrial complex I and their defects in diseaseQ37765531
Supramolecular organizations in the aerobic respiratory chain of Escherichia coliQ39480857
The random collision model and a critical assessment of diffusion and collision in mitochondrial electron transportQ39505283
Variation of flux control coefficient of cytochrome c oxidase and of the other respiratory chain complexes at different values of protonmotive force occurs by a threshold mechanism.Q39544308
Respiratory active mitochondrial supercomplexes.Q39913363
Mitochondrial DNA background modulates the assembly kinetics of OXPHOS complexes in a cellular model of mitochondrial disease.Q39937507
An assembled complex IV maintains the stability and activity of complex I in mammalian mitochondriaQ40142379
Respiratory chain supercomplexes set the threshold for respiration defects in human mtDNA mutant cybridsQ40272913
Cellular pathophysiological consequences of BCS1L mutations in mitochondrial complex III enzyme deficiency.Q43047012
A novel nonsense mutation (Q352X) in the mitochondrial cytochrome b gene associated with a combined deficiency of complexes I and III.Q43814295
Identification and characterization of a common set of complex I assembly intermediates in mitochondria from patients with complex I deficiencyQ44588150
Significance of respirasomes for the assembly/stability of human respiratory chain complex I.Q47316196
Novel mutations in the NDUFS1 gene cause low residual activities in human complex I deficienciesQ48240631
Human mitochondrial complex I assembles through the combination of evolutionary conserved modules: a framework to interpret complex I deficiencies.Q50488580
Combined OXPHOS complex I and IV defect, due to mutated complex I assembly factor C20ORF7Q56592348
Novel insights into the assembly and function of human nuclear-encoded cytochromecoxidase subunits 4, 5a, 6a, 7a and 7bQ57847623
A Simple and Rapid Assay of Oxidative PhosphorylationQ59068452
Assembly of nuclear DNA-encoded subunits into mitochondrial complex IV, and their preferential integration into supercomplex forms in patient mitochondriaQ63359727
Combined enzymatic complex I and III deficiency associated with mutations in the nuclear encoded NDUFS4 geneQ74177562
Early-onset multisystem mitochondrial disorder caused by a nonsense mutation in the mitochondrial DNA cytochrome C oxidase II geneQ74518028
Mitochondrial complex III stabilizes complex I in the absence of NDUFS4 to provide partial activityQ85030067
P433issue3
P304page(s)324-335
P577publication date2012-02-16
P1433published inCell MetabolismQ1254684
P1476titleMitochondrial complex I plays an essential role in human respirasome assembly
P478volume15

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