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Echaniz-Laguna 2002 Ann Neurol

From Bioblast
Publications in the MiPMap
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

MitoEAGLE VO2max/BME database

  • Human vastus lateralis
  • 1 female & 6 males
  • 55 years
  • Truely sedentary control group
  • H
  • M
  • BME
  • BMI = 29.0 kg·m-2
  • VO2max/M = 29.5 mL·min-1·kg-1 (= VO2peak/M/0.93)
  • Permeabilized muscle fibres; 22 °C; GMP; md; conversions: Gnaiger 2009 Int J Biochem Cell Biol
  • JO2,P(NS) = 56.9 µmol·s-1·kg-1 wet muscle mass (37 °C)
  • JO2,P(GM) = 33.7 µmol·s-1·kg-1 wet muscle mass (37 °C)
  • JO2,P(NS) = JO2,P(GM)/0.73
  • Fiber wet mass to dry mass ratio = 3.5 (N'Guessan 2004 Mol Cell Biochem)

References: BME and VO2max

» VO2max
Bakkman 2007 ActaPhysiolBakkman L, Sahlin K, Holmberg HC, Tonkonogi M (2007) Quantitative and qualitative adaptation of human skeletal muscle mitochondria to hypoxic compared with normoxic training at the same relative work rate. Acta Physiol (Oxford) 190:243–51.
Boushel 2007 DiabetologiaBoushel RC, Gnaiger E, Schjerling P, Skovbro M, Kraunsoee R, Dela F (2007) Patients with Type 2 diabetes have normal mitochondrial function in skeletal muscle. Diabetologia 50:790-6.
Chambers 2020 J Appl Physiol (1985)Chambers TL, Burnett TR, Raue U, Lee GA, Finch WH, Graham BM, Trappe TA, Trappe S (2020) Skeletal muscle size, function, and adiposity with lifelong aerobic exercise. J Appl Physiol (1985) 128:368–78.
Daussin 2008 Am J Physiol Regul Integr Comp PhysiolDaussin FN, Zoll J, Dufour SP, Ponsot E, Lonsdorfer-Wolf E, Doutreleau S, Mettauer B, Piquard F, Geny B, Richard R (2008) Effect of interval versus continuous training on cardiorespiratory and mitochondrial functions: relationship to aerobic performance improvements in sedentary subjects. Am J Physiol Regul Integr Comp Physiol 295:R264-72.
Garnier 2005 FASEB JGarnier A, Fortin D, Zoll J, N'Guessan B, Mettauer B, Lampert E, Veksler V, Ventura-Clapier R (2005) Coordinated changes in mitochondrial function and biogenesis in healthy and diseased human skeletal muscle. FASEB J 19:43-52.
Gnaiger 2015 Scand J Med Sci SportsGnaiger E, Boushel R, Søndergaard H, Munch-Andersen T, Damsgaard R, Hagen C, Díez-Sánchez C, Ara I, Wright-Paradis C, Schrauwen P, Hesselink M, Calbet JAL, Christiansen M, Helge JW, Saltin B (2015) Mitochondrial coupling and capacity of oxidative phosphorylation in skeletal muscle of Inuit and caucasians in the arctic winter.
Gnaiger 2019 MiP2019
Erich Gnaiger
OXPHOS capacity in human muscle tissue and body mass excess – the MitoEAGLE mission towards an integrative database (Version 6; 2020-01-12).
Loe 2013 PLOS ONELoe H, Rognmo Ø, Saltin B, Wisløff U (2013) Aerobic capacity reference data in 3816 healthy men and women 20-90 years. PLOS ONE 8:e64319.
Mettauer 2001 J Am Coll CardiolMettauer B, Zoll J, Sanchez H, Lampert E, Ribera F, Veksler V, Bigard X, Mateo P, Epailly E, Lonsdorfer J, Ventura-Clapier R (2001) Oxidative capacity of skeletal muscle in heart failure patients versus sedentary or active control subjects. J Am Coll Cardiol 38:947-54.
Mogensen 2006 J PhysiolMogensen M, Bagger M, Pedersen PK, Fernström M, Sahlin K (2006) Cycling efficiency in humans is related to low UCP3 content and to type I fibres but not to mitochondrial efficiency. J Physiol 571:669-81.
N'Guessan 2004 Mol Cell BiochemN'Guessan B, Zoll J, Ribera F, Ponsot E, Lampert E, Ventura-Clapier R, Veksler V, Mettauer B (2004) Evaluation of quantitative and qualitative aspects of mitochondrial function in human skeletal and cardiac muscles. Mol Cell Biochem 256-257:267-80.
Pesta 2011 Am J Physiol Regul Integr Comp PhysiolPesta D, Hoppel F, Macek C, Messner H, Faulhaber M, Kobel C, Parson W, Burtscher M, Schocke M, Gnaiger E (2011) Similar qualitative and quantitative changes of mitochondrial respiration following strength and endurance training in normoxia and hypoxia in sedentary humans. Am J Physiol Regul Integr Comp Physiol 301:R1078–87.
Ponsot 2006 J Appl Physiol (1985)Ponsot E, Dufour SP, Zoll J, Doutrelau S, N'Guessan B, Geny B, Hoppeler H, Lampert E, Mettauer B, Ventura-Clapier R, Richard R (2006) Exercise training in normobaric hypoxia in endurance runners. II. Improvement of mitochondrial properties in skeletal muscle. J Appl Physiol (1985) 100:1249-57.
Pribis 2010 NutrientsPribis P, Burtnack CA, McKenzie SO, Thayer J (2010) Trends in body fat, body mass index and physical fitness among male and female college students. Nutrients 2:1075-85.
Raboel 2009 Diabetes Obes MetabRaboel R, Hojberg PM, Almdal T, Boushel RC, Haugaard SB, Madsbad S, Dela F (2009) Improved glycaemic control decreases inner mitochondrial membrane leak in type 2 diabetes. Diabetes Obes Metab 11:355-60.
Rasmussen 2001 Am J Physiol Endocrinol MetabRasmussen UF, Rasmussen HN, Krustrup P, Quistorff B, Saltin B, Bangsbo J (2001) Aerobic metabolism of human quadriceps muscle: in vivo data parallel measurements on isolated mitochondria. Am J Physiol Endocrinol Metab 280:E301-7.
Rasmussen 2003 Eur J PhysiolRasmussen UF, Krustrup P, Kjaer M, Rasmussen HN (2003) Human skeletal muscle mitochondrial metabolism in youth and senescence: no signs of functional changes in ATP formation and mitochondrial oxidative capacity. Pflugers Arch – Eur J Physiol 446:270-78.
Zoll 2002 J PhysiolZoll J, Sanchez H, N'Guessan B, Ribera F, Lampert E, Bigard X, Surrurier B, Fortin D, Geny B, Veksler V, Ventura-Clapier R, Mettauer B (2002) Physical activity changes the regulation of mitochondrial respiration in human skeletal muscle. J Physiol 543:191-200.

Labels: MiParea: Respiration  Pathology: Neurodegenerative 

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

Coupling state: LEAK, OXPHOS  Pathway: N, S