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rdf:type |
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lifeskim:mentions |
umls-concept:C0024501,
umls-concept:C0024660,
umls-concept:C0026237,
umls-concept:C0521346,
umls-concept:C0721534,
umls-concept:C1176474,
umls-concept:C1514873,
umls-concept:C1546857,
umls-concept:C1556066,
umls-concept:C1619636,
umls-concept:C2245017
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pubmed:issue |
6
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pubmed:dateCreated |
2004-3-31
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pubmed:abstractText |
A puzzling observation in patients with oxidative phosphorylation (OXPHOS) deficiencies is the presence of combined enzyme complex defects associated with a genetic alteration in only one protein-coding gene. In particular, mutations in the mtDNA encoded cytochrome b gene are associated either with combined complex I+III deficiency or with only complex III deficiency. We have reproduced the combined complex I+III defect in mouse and human cultured cell models harboring cytochrome b mutations. In both, complex III assembly is impeded and causes a severe reduction in the amount of complex I, not observed when complex III activity was pharmacologically inhibited. Metabolic labeling in mouse cells revealed that complex I was assembled, although its stability was severely hampered. Conversely, complex III stability was not influenced by the absence of complex I. This structural dependence among complexes I and III was confirmed in a muscle biopsy of a patient harboring a nonsense cytochrome b mutation.
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pubmed:grant |
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-10231371,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-10502593,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-10775262,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-10944442,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-11047755,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-11073949,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-11079541,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-11112787,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-11262412,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-11381606,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-11381612,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-11528392,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-11731284,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-11798023,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-12554686,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-12709789,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-12758076,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-12826641,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-12941961,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-12954771,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-14506725,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-2814477,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-6309810,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-7221838,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-8393806,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-8638158,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-8643997,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-8738332,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-8965693,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-8965720,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-8965726,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-9037024,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-9651245,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-9707444,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-9818877,
http://linkedlifedata.com/resource/pubmed/commentcorrection/15053874-9837813
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Mar
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pubmed:issn |
1097-2765
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:day |
26
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pubmed:volume |
13
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
805-15
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pubmed:dateRevised |
2011-9-13
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pubmed:meshHeading |
pubmed-meshheading:15053874-Animals,
pubmed-meshheading:15053874-Cells, Cultured,
pubmed-meshheading:15053874-Clone Cells,
pubmed-meshheading:15053874-Codon, Nonsense,
pubmed-meshheading:15053874-Cytochromes b,
pubmed-meshheading:15053874-DNA, Mitochondrial,
pubmed-meshheading:15053874-Electron Transport Complex III,
pubmed-meshheading:15053874-Humans,
pubmed-meshheading:15053874-L Cells (Cell Line),
pubmed-meshheading:15053874-Mammals,
pubmed-meshheading:15053874-Mice,
pubmed-meshheading:15053874-Mitochondria,
pubmed-meshheading:15053874-Models, Molecular,
pubmed-meshheading:15053874-Mutagenesis, Site-Directed,
pubmed-meshheading:15053874-Mutation, Missense,
pubmed-meshheading:15053874-Oxidative Phosphorylation
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pubmed:year |
2004
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pubmed:articleTitle |
Respiratory complex III is required to maintain complex I in mammalian mitochondria.
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pubmed:affiliation |
Departamento de Bioquímica y Biología Molecular y Celular, Universidad de Zaragoza, Miguel Servet, 177, Zaragoza 50013, Spain.
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pubmed:publicationType |
Journal Article
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