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Muscular mitochondr...
Muscular mitochondrial function in amyotrophic lateral sclerosis is progressively altered as the disease develops : A temporal study in man
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- Echaniz-Laguna, Andoni (författare)
- Département de Neurologie, Hopital Civil de Strasbourg, 1 Place de l’Hopital, Strasbourg, France; INSERM U-692, Faculté de Médecine, Strasbourg, France
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- Zoll, Joffrey (författare)
- Département de Physiologie, Faculté de Médecine, Strasbourg, France
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- Ponsot, Elodie, 1973- (författare)
- Département de Physiologie, Faculté de Médecine, Strasbourg, France
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- N'guessan, Benoit (författare)
- Département de Physiologie, Faculté de Médecine, Strasbourg, France
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- Tranchant, Christine (författare)
- Département de Neurologie, Hopital Civil de Strasbourg, 1 Place de l’Hospital, Strasbourg, France
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- Loeffler, Jean-Philippe (författare)
- INSERM U-692, Faculté de Médecine, Strasbourg, France
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- Lampert, Eliane (författare)
- Département de Physiologie, Faculté de Médecine, Strasbourg, France
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(creator_code:org_t)
- San Diego, USA : Elsevier, 2006
- 2006
- Engelska.
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Ingår i: Experimental Neurology. - San Diego, USA : Elsevier. - 0014-4886 .- 1090-2430. ; 198:1, s. 25-30
- Relaterad länk:
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
Ämnesord
Stäng
- 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.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Medicinska och farmaceutiska grundvetenskaper -- Fysiologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Basic Medicine -- Physiology (hsv//eng)
Nyckelord
- Mitochondria
- muscle
- amyotrophic lateral sclerosis
Publikations- och innehållstyp
- ref (ämneskategori)
- art (ämneskategori)
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