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Mitochondrial respiratory chain function in skeletal muscle of ALS patients
Author(s) -
EchanizLaguna Andoni,
Zoll Joffrey,
Ribera Florence,
Tranchant Christine,
Warter JeanMarie,
Lonsdorfer Jean,
Lampert Eliane
Publication year - 2002
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.10357
Subject(s) - amyotrophic lateral sclerosis , mitochondrion , skeletal muscle , sed , medicine , biology , endocrinology , mitochondrial respiratory chain , respiratory chain , mitochondrial disease , oxidative phosphorylation , mitochondrial dna , biochemistry , disease , gene
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, μmol 0 2 · min −1 · gm −1 dw and V max : SALS, 3.1 ± 0.3; SED, 2.5 ± 0.3, μmol 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.