| Standardized mitochondrial analysis gives new insights into mitochondrial dynamics and OPA1 function. | |
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MedLine Citation:
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PMID: 22433900 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Mitochondria form dynamic tubular networks through processes of fission and fusion. Defect in mitochondrial dynamics lead to various pathologies, including several common and some rare neurodegenerative disorders. OPA1 and MFN2 are two key players in mitochondrial fusion associated with Autosomal Dominant Optic Atrophy and Charcot Marie Tooth neuropathy type 2A respectively. We used micropatterned coverslips to standardize the visualization of mitochondrial distribution in skin fibroblasts. In fibroblasts from affected patients, mutations in the OPA1 and MFN2 genes were found to affect the volume and cellular distribution of mitochondria. In G1/S cell cycle phase, mitochondria emerging from the microtubule organizing centre may be crucial to mitochondrial biogenesis since it appeared to be protected against mitochondrial fragmentation induced by OPA1 mutations. The standardized quantitative analysis of the mitochondrial network and the description of mitochondrial subcellular distribution should lead to better diagnostic criteria for mitochondrial diseases and yield new insights into mitochondrial dysfunction in disease and aging. |
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Authors:
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Arnaud Chevrollier; Julien Cassereau; Marc Ferre; Jennifer Alban; Valérie Desquiret-Dumas; Naïg Gueguen; Patrizia Amati-Bonneau; Vincent Procaccio; Dominique Bonneau; Pascal Reynier |
Publication Detail:
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Type: JOURNAL ARTICLE Date: 2012-3-17 |
Journal Detail:
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Title: The international journal of biochemistry & cell biology Volume: - ISSN: 1878-5875 ISO Abbreviation: - Publication Date: 2012 Mar |
Date Detail:
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Created Date: 2012-3-21 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9508482 Medline TA: Int J Biochem Cell Biol Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
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Copyright © 2012. Published by Elsevier Ltd. |
Affiliation:
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CNRS 6214, Angers, F-49000, France; INSERM U771, Angers, F-49000, France; Centre Hospitalier Universitaire Département de Biochimie et Génétique, Angers, F-49000 France; Université LUNAM, Angers, F-49000, France. |
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