title: Mitochondrial respiratory chain function in skeletal muscle of ALS patients.
abstract: Evidence implicating mitochondrial dysfunction in the central nervous system of patients with sporadic amyotrophic lateral sclerosis (SALS) has recently been accumulating. In contrast, data on mitochondrial function in skeletal muscle in SALS are scarce and controversial. We investigated the in situ properties of muscle mitochondria in patients with early-stage SALS and sedentary (SED) controls using the skinned fiber technique to determine whether respiration of muscle tissue is altered in early-stage SALS in comparison with SED. Musculus vastus lateralis biopsies were obtained from 7 SED group members and 14 patients with early-stage SALS (mean disease duration, 9 months). Muscle fibers were permeabilized with saponine and then skinned and placed in an oxygraphic chamber to measure basal (V(0)) and maximal (V(max)) adenosine diphosphate-stimulated respiration rates and to assess mitochondrial regulation by adenosine diphosphate. Muscle oxidative capacity, evaluated with V(max), was identical in patients in the SALS and SED groups (V(0): SALS, 1.1 +/- 0.1; SED, 0.8 +/- 0.1, micromol 0(2). min(-1). gm(-1)dw and V(max): SALS, 3.1 +/- 0.3; SED, 2.5 +/- 0.3, micromol 0(2). min(-1). gm(-1)dw). This study shows an absence of large mitochondrial damage in skeletal muscle of patients with early-stage SALS, suggesting that mitochondrial dysfunction in the earlier stages of SALS is almost certainly not systemic.
authors.affiliation: Service des Maladies du Système Nerveux et du Muscle, Clinique Neurologique, Hôpital Civil de Strasbourg, 1 Place de l'Hôpital, BP 426, 67091 Strasbourg, France. echaniz-laguna@medecine.u-strasbg.fr
authors.forename: Andoni
authors.initials: A
authors.lastname: Echaniz-Laguna
authors.forename: Joffrey
authors.initials: J
authors.lastname: Zoll
authors.forename: Florence
authors.initials: F
authors.lastname: Ribera
authors.forename: Christine
authors.initials: C
authors.lastname: Tranchant
authors.forename: Jean-Marie
authors.initials: JM
authors.lastname: Warter
authors.forename: Jean
authors.initials: J
authors.lastname: Lonsdorfer
authors.forename: Eliane
authors.initials: E
authors.lastname: Lampert
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000378:metabolism*; D035061:Control Groups; D004579:Electron Transport / Q000502:physiology; D005080:Exercise Test; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D008931:Mitochondria, Muscle / Q000378:metabolism*; D018482:Muscle, Skeletal / Q000378:metabolism*