Differences in assembly or stability of complex I and other mitochondrial OXPHOS complexes in inherited complex I deficiency
about
Decreased intracellular ATP content and intact mitochondrial energy generating capacity in human cystinotic fibroblastsA molecular chaperone for mitochondrial complex I assembly is mutated in a progressive encephalopathy.Oxidative stress in cancer associated fibroblasts drives tumor-stroma co-evolution: A new paradigm for understanding tumor metabolism, the field effect and genomic instability in cancer cellsHuman CIA30 is involved in the early assembly of mitochondrial complex I and mutations in its gene cause diseaseAnalysis of the assembly profiles for mitochondrial- and nuclear-DNA-encoded subunits into complex ICytochrome c oxidase is required for the assembly/stability of respiratory complex I in mouse fibroblastsMice with mitochondrial complex I deficiency develop a fatal encephalomyopathy.Supramolecular organization of the respiratory chain in Neurospora crassa mitochondriaInvestigation of the role of mitochondrial DNA in multiple sclerosis susceptibility.Mitochondrial disorders of the nuclear genomeCoexpression within Integrated Mitochondrial Pathways Reveals Different Networks in Normal and Chemically Treated Transcriptomes.Structure of a mitochondrial supercomplex formed by respiratory-chain complexes I and IIIMitochondrial complex I deficiency enhances skeletal myogenesis but impairs insulin signaling through SIRT1 inactivation.The p.M292T NDUFS2 mutation causes complex I-deficient Leigh syndrome in multiple familiesDJ-1 null dopaminergic neuronal cells exhibit defects in mitochondrial function and structure: involvement of mitochondrial complex I assemblyAltered mitochondrial function and oxidative stress in leukocytes of anorexia nervosa patients.The respiratory chain supercomplex organization is independent of COX7a2l isoforms.Pioglitazone leads to an inactivation and disassembly of complex I of the mitochondrial respiratory chainRespiratory chain complex I deficiency caused by mitochondrial DNA mutations.Insights into the composition and assembly of the membrane arm of plant complex I through analysis of subcomplexes in Arabidopsis mutant lines.A mutation screening of oncogenes, tumor suppressor gene TP53 and nuclear encoded mitochondrial complex I genes in oncocytic thyroid tumors.Hypothalamic mitochondrial dysfunction associated with anorexia in the anx/anx mouse.Mitochondrial complex I plays an essential role in human respirasome assembly.Diabetes Susceptibility Genes Pdx1 and Clec16a Function in a Pathway Regulating Mitophagy in β-Cells.Novel MTND1 mutations cause isolated exercise intolerance, complex I deficiency and increased assembly factor expression.Mouse models of mitochondrial complex I dysfunctionMitochondrial respiratory complex I: structure, function and implication in human diseases.An immunohistochemical procedure to detect patients with paraganglioma and phaeochromocytoma with germline SDHB, SDHC, or SDHD gene mutations: a retrospective and prospective analysis.Functional diversity of complex I subunits in Candida albicans mitochondria.Human NADH:ubiquinone oxidoreductase deficiency: radical changes in mitochondrial morphology?Alzheimer's brains harbor somatic mtDNA control-region mutations that suppress mitochondrial transcription and replicationLack of complex I is associated with oncocytic thyroid tumoursMitochondrial-nuclear interactions: compensatory evolution or variable functional constraint among vertebrate oxidative phosphorylation genes?Discovering genes involved in alcohol dependence and other alcohol responses: role of animal models.Mouse models for nuclear DNA-encoded mitochondrial complex I deficiency.Whole Exome Sequencing Identifies the Genetic Basis of Late-Onset Leigh Syndrome in a Patient with MRI but Little Biochemical Evidence of a Mitochondrial Disorder.Cellular and animal models for mitochondrial complex I deficiency: a focus on the NDUFS4 subunit.Leigh syndrome in Drosophila melanogaster: morphological and biochemical characterization of Surf1 post-transcriptional silencingPost-Translational Oxidative Modifications of Mitochondrial Complex I (NADH: Ubiquinone Oxidoreductase): Implications for Pathogenesis and Therapeutics in Human Diseases.The antioxidant Trolox restores mitochondrial membrane potential and Ca2+ -stimulated ATP production in human complex I deficiency.
P2860
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P2860
Differences in assembly or stability of complex I and other mitochondrial OXPHOS complexes in inherited complex I deficiency
description
2004 nî lūn-bûn
@nan
2004 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
2004 թվականի մարտին հրատարակված գիտական հոդված
@hy
2004年の論文
@ja
2004年論文
@yue
2004年論文
@zh-hant
2004年論文
@zh-hk
2004年論文
@zh-mo
2004年論文
@zh-tw
2004年论文
@wuu
name
Differences in assembly or sta ...... inherited complex I deficiency
@ast
Differences in assembly or sta ...... inherited complex I deficiency
@en
Differences in assembly or sta ...... inherited complex I deficiency
@en-gb
Differences in assembly or sta ...... inherited complex I deficiency
@nl
type
label
Differences in assembly or sta ...... inherited complex I deficiency
@ast
Differences in assembly or sta ...... inherited complex I deficiency
@en
Differences in assembly or sta ...... inherited complex I deficiency
@en-gb
Differences in assembly or sta ...... inherited complex I deficiency
@nl
prefLabel
Differences in assembly or sta ...... inherited complex I deficiency
@ast
Differences in assembly or sta ...... inherited complex I deficiency
@en
Differences in assembly or sta ...... inherited complex I deficiency
@en-gb
Differences in assembly or sta ...... inherited complex I deficiency
@nl
P2093
P921
P356
P1476
Differences in assembly or sta ...... inherited complex I deficiency
@en
P2093
Cristina Ugalde
Jan A M Smeitink
Leo G J Nijtmans
Rolf J R J Janssen
P304
P356
10.1093/HMG/DDH071
P407
P577
2004-03-15T00:00:00Z