| Mitochondrial respiratory chain complex assembly and function during human fetal development. | |
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MedLine Citation:
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PMID: 18249146 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Oxidative phosphorylation (OXPHOS) deficiency may have early antenatal manifestations, probably related to the time course and/or tissue specificity of the disease gene expression during the embryo-fetal period. This feature hampers a fully reliable prenatal enzymological diagnosis of OXPHOS deficiency. We have studied OXPHOS in various human fetal tissues from 9 to 17 weeks of gestation. We found that the fetal respiratory chain complexes are fully assembled and functional at early stages of development in heart, liver, muscle, brain and kidney. We also observed a marked increase of respiratory chain activities and mitochondria content in postnatal compared to prenatal tissues. However, we were not able to detect obvious modification in the size, composition or activity of the various OXPHOS complexes during the second trimester of pregnancy that could account for the variations we observed in a pathological context. Therefore, we suggest that the time-dependent expression of respiratory chain deficiency either during fetal life or after birth could be related to the differential expression or regulation of the mutant proteins. |
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Authors:
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Limor Minai; Jelena Martinovic; Dominique Chretien; Françoise Dumez; Férechté Razavi; Arnold Munnich; Agnès Rötig |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2008-01-30 |
Journal Detail:
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Title: Molecular genetics and metabolism Volume: 94 ISSN: 1096-7206 ISO Abbreviation: Mol. Genet. Metab. Publication Date: 2008 May |
Date Detail:
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Created Date: 2008-04-21 Completed Date: 2008-07-21 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9805456 Medline TA: Mol Genet Metab Country: United States |
Other Details:
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Languages: eng Pagination: 120-6 Citation Subset: IM |
Affiliation:
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INSERM U781 and Service de Génétique, Hôpital Necker-Enfants Malades, 149 rue de Sèvres, 75015 Paris, France. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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DNA, Mitochondrial
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analysis Electron Transport Electron Transport Chain Complex Proteins / metabolism* Electron Transport Complex I / metabolism Electron Transport Complex II / metabolism Electron Transport Complex III / metabolism Electron Transport Complex IV / metabolism Female Fetal Development / physiology* Fetus / embryology Humans Mitochondria / enzymology, metabolism* Mitochondrial Diseases / metabolism Mitochondrial Proteins / metabolism Oxidative Phosphorylation Pregnancy |
| Chemical | |
Reg. No./Substance:
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0/DNA, Mitochondrial; 0/Electron Transport Chain Complex Proteins; 0/Mitochondrial Proteins; EC 1.10.2.2/Electron Transport Complex III; EC 1.3.5.1/Electron Transport Complex II; EC 1.6.5.3/Electron Transport Complex I; EC 1.9.3.1/Electron Transport Complex IV |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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