Echaniz-Laguna 2002 Ann Neurol

Echaniz-Laguna A, Zoll J, Ribera F, Tranchant C, Warter JM, Lonsdorfer J, Lampert E (2002) Mitochondrial respiratory chain function in skeletal muscle of ALS patients. Ann Neurol 52:623-7.

» PMID: 12402260

Echaniz-Laguna A, Zoll J, Ribera F, Tranchant C, Warter JM, Lonsdorfer J, Lampert E (2002) Ann Neurol

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.

• Bioblast editor: Gnaiger E

Labels: MiParea: Respiration  Pathology: Neurodegenerative 

Organism: Human  Tissue;cell: Skeletal muscle  Preparation: Intact organism, Permeabilized tissue 

Coupling state: LEAK, OXPHOS  Pathway: N, S 

MitoEAGLE BME 

MitoEAGLE V_O2max/BME data base

• Human vastus lateralis
• 1 female & 6 males
• 55 years
• Truely sedentary control group
• h
• m
• BME
• BMI = 29.0 kg·m^-2
• V_O2max/BM = 29.5 mL·min^-1·kg^-1 (= V_O2peak/BM/0.93)
• Permeabilized muscle fibres; 22 °C; GM_P; m_d; conversions: Gnaiger 2009 Int J Biochem Cell Biol
• J_O2,P(NS) = 56.9 µmol·s^-1·kg^-1 wet muscle mass (37 °C)

• J_O2,P(GM) = 33.7 µmol·s^-1·kg^-1 wet muscle mass (37 °C)
• J_O2,P(NS) = J_O2,P(GM)/0.73
• Fiber wet mass to dry mass ratio = 3.5 (N'Guessan 2004 Mol Cell Biochem)

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