Muscular mitochondrial function in amyotrophic lateral sclerosis is progressively altered as the disease develops: a temporal study in man.

Date publication

mars 2006

Auteurs

Membres identifiés du Cancéropôle Est :
Dr LOEFFLER Jean-Philippe

Tous les auteurs :
Echaniz-Laguna A, Zoll J, Ponsot E, N'guessan B, Tranchant C, Loeffler JP, Lampert E

Lien Pubmed

https://www.ncbi.nlm.nih.gov/pubmed/16126198

Résumé

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.

Référence

Exp Neurol. 2006 Mar;198(1):25-30