TY - JOUR
T1 - Muscular mitochondrial function in amyotrophic lateral sclerosis is progressively altered as the disease develops
T2 - A temporal study in man
AU - Echaniz-Laguna, Andoni
AU - Zoll, Joffrey
AU - Ponsot, Elodie
AU - N'Guessan, Benoit
AU - Tranchant, Christine
AU - Loeffler, Jean Philippe
AU - Lampert, Eliane
PY - 2006/3
Y1 - 2006/3
N2 - 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.
AB - 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.
KW - Amyotrophic lateral sclerosis
KW - Mitochondria
KW - Muscle
UR - http://www.scopus.com/inward/record.url?scp=32644434546&partnerID=8YFLogxK
U2 - 10.1016/j.expneurol.2005.07.020
DO - 10.1016/j.expneurol.2005.07.020
M3 - Article
C2 - 16126198
AN - SCOPUS:32644434546
SN - 0014-4886
VL - 198
SP - 25
EP - 30
JO - Experimental Neurology
JF - Experimental Neurology
IS - 1
ER -