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Muscular mitochondrial function in amyotrophic lateral sclerosis is progressively altered as the disease develops: A temporal study in man
Département de Neurologie, Hopital Civil de Strasbourg, 1 Place de l’Hopital, Strasbourg, France; INSERM U-692, Faculté de Médecine, Strasbourg, France.
Département de Physiologie, Faculté de Médecine, Strasbourg, France.
Département de Physiologie, Faculté de Médecine, Strasbourg, France.ORCID iD: 0000-0002-8071-4745
Département de Physiologie, Faculté de Médecine, Strasbourg, France.
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2006 (English)In: Experimental Neurology, ISSN 0014-4886, E-ISSN 1090-2430, Vol. 198, no 1, 25-30 p.Article in journal (Refereed) Published
Abstract [en]

We performed repeated analysis of mitochondrial respiratory function in skeletal muscle (SM) of patients with early-stage sporadic amyotrophic lateral sclerosis (SALS) to determine whether mitochondrial function was altered as the disease advanced. SM biopsies were obtained from 7 patients with newly diagnosed SALS, the same 7 patients 3 months later, and 7 sedentary controls. Muscle fibers were permeabilized with saponin, then skinned and placed in an oxygraphic chamber to measure basal and maximal adenosine diphosphate (ADP)-stimulated respiration rates and to assess mitochondrial regulation by ADP. We found that the maximal oxidative phosphorylation capacity of muscular mitochondria significantly increased, and muscular mitochondrial respiratory complex IV activity significantly decreased as the disease advanced. This temporal study demonstrates for the first time that mitochondrial function in SM in human SALS is progressively altered as the disease develops.

Place, publisher, year, edition, pages
San Diego, USA: Elsevier, 2006. Vol. 198, no 1, 25-30 p.
Keyword [en]
Mitochondria, muscle, amyotrophic lateral sclerosis
National Category
Medical and Health Sciences Physiology
Identifiers
URN: urn:nbn:se:oru:diva-43580DOI: 10.1016/j.expneurol.2005.07.020ISI: 000235747000004PubMedID: 16126198Scopus ID: 2-s2.0-32644434546OAI: oai:DiVA.org:oru-43580DiVA: diva2:794995
Available from: 2015-03-13 Created: 2015-03-13 Last updated: 2015-04-02Bibliographically approved

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