Skip to main content
SpringerLink
Log in

Einfluß der mitochondrialen Radikalproduktion des Skelettmuskels auf die Pathogenese der Duchenne-Muskeldystrophie (mdx-Maus Modell) unter Berücksichtigung der Auswirkungen einer submaximalen physischen Belastung

  • Conference paper
Oxidativer Stress in der Kinderheilkunde

  • 27 Accesses

Zusammenfassung

Die progressiv verlaufende Muskeldystrophie vom Typ Duchenne basiert auf einer x-chromosomalen Deletion im Bereich der Xp21-Region (Kunkel 1986). Infolge dieser Deletion unterbleibt die Synthese des Dystrophins, eines membranstabilisierenden Proteins (Hoffman et al. 1987).

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 54.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 69.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Literatur

  • Austin L (1990) How the lack of dystrophin may upset calcium regulation and lead to oxidative damage. In: Kakulas BA, Mastaglia FL (eds) Pathogenesis and therapy of Duchenne and Becker muscular dystrophy. Raven Press, New York, pp 69–75

    Google Scholar 

  • Austin L, Niese MD, McGregor A et al. (1992) Potential oxyradical damage and energy status in individual muscle fibres from degenerating muscle diseases. Neuromusc Disord 2 (1)127–33

    Article  Google Scholar 

  • Barth PG, Schölte HR, Berden JA et al. (1983) An x-linked mitochondrial disease affecting cardiac muscle, skeletal muscle and neutrophil leukocytes. 1J Neurol Sci 62:327–355

    Article  CAS  Google Scholar 

  • Beutler E (1984) Glutathion peroxidase. In: Beutler E (ed) Red Cell Metabolism. A mannual of biochemical methods. Grune & Straton, New York 1974

    Google Scholar 

  • Byrne E, Dennett X (1992) Respiratory chain failure in adult muscle fibres: relationship with ageing and possible implications for the neuronal pool. Mut Res 275:125–131

    CAS  Google Scholar 

  • Chance B, Williams CM (1956) The respiratory chain and oxidative phosphorylation. Adv Enzymol 17:65–134

    CAS  Google Scholar 

  • Deutsche Gesellschaft für Klinische Chemie (1977) Standardmethode zur Bestimmung der Aktivität der Creatinkinase. J Clin Chem Biochem 15:249–254

    Google Scholar 

  • Dunn JF, Frostick S, Brown G, Radda GK (1991) Energy status of cells lacking dystrophin: an in vivo/in vitro study of mdx mouse skeletal muscle. Biochim Biophys Acta 1096:115–120

    PubMed  CAS  Google Scholar 

  • Emery AEH (1987) Duchenne muscular dystrophy. Oxford Monographs on Medical Genetics. Oxford University Press, Oxford

    Google Scholar 

  • Faist V, König J, Höger H, Elmadfa I (1992) Das Modell der mdx-Maus: Auswirkungen des genetischen Defektes auf Plasma- und Muskelaktivitäten verschiedener Muskelenzyme unter physischer Belastung. 30. Wissenschaftliche Tagung der GV-SOLAS, Salzburg

    Google Scholar 

  • Fujii H, Miwa S (1987) Pyruvate Kinase. In: Bergmeyer HU (ed) Methods of enzymatic analysis, vol III. Verlag Chemie, Weinheim, pp 496–501

    Google Scholar 

  • Gains RF, Pueschel SM, Sassman EA, Driscoll J (1982) Effect of exercise on serum creatine kinase in carriers of Duchenne muscular dystrophy. J Med Genetics 19:4–7

    Article  Google Scholar 

  • Glazer, A (1990) Phycoerythrin fluorescence-based assay for reactive oxygen species. Methods in Enzymology 186:161–167

    Article  PubMed  CAS  Google Scholar 

  • Halliwell B, Gutterdige JMC (1989) Free radicals in biology and medicine, 2nd edn. Oxford University Press, Oxford

    Google Scholar 

  • Hammer C (1988) Verteilung und Akkumulation autofluoreszierender Pigmente in Teleostien und deren Bestimmungsgrößen. Dissertation, Universität Hamburg

    Google Scholar 

  • Hoffman EP, Brown RH, Kunkel LM (1987) Dystrophin: the protein product of the Duchenne muscular dystrophy locus. Cell 51:919–928

    Article  PubMed  CAS  Google Scholar 

  • Jackson MJ, Jones DA, Edwards RHT (1984) Techniques for studying free radical damage in muscular dystrophy. Med Biol 62:135–138

    PubMed  CAS  Google Scholar 

  • Kikugawa K (1986) Fluorescent products derived from the reaction of primary amines and components in peroxidized lipids. Adv Free Radic Biol Med 2:389–417

    Article  CAS  Google Scholar 

  • Kunkel LM (1986) Analyses of deletions in DNA from patients with Becker and Duchenne muscular dystrophy. Nature 322:73–77

    Article  PubMed  CAS  Google Scholar 

  • Lowry H, Rosebrough NJ, Farr AL, Randall RJ (1951) Protein measurement with the folin phenol reagent. J Biol Chem 193:265–275

    PubMed  CAS  Google Scholar 

  • Maguire JJ, Kagan V, Ackrell BAC et al. (1992) Succinate-ubichinone reductase linked recycling of aaa-tocopherol in reconstituted systems and mitochondria: Requirement for reduced ubichinone. Arch Biochem Biophys 292 (I):47–53

    Article  PubMed  CAS  Google Scholar 

  • Marklund S, Marklund G (1974) Involvement of the superoxide anion radical in the autoxidation of pyrogallol and convenient assay for superoxide dismutase. Eur J Biochem 47:469–474

    Article  PubMed  CAS  Google Scholar 

  • Murphy MP (1989) Slip and leak in mitochondrial oxidative phosphorylation. Biochim Biophys Acta 977:143–141

    Google Scholar 

  • Nicholls P, Buttko P (1993) Protons, pumps and potentials: Control of cytochrome c oxidase. J Bioen Biomem 25 (2):533–539

    Google Scholar 

  • Packer L, Slater TF, Willson RL (1979) Direct observation of a free radical interaction between vitamin E and vitamin C. Nature 278:737–738

    Article  PubMed  CAS  Google Scholar 

  • Rickwood D, Wilson MT, Darley-Usmar M (1987) Isolation and characteristics of intact mitochondria. In: Darley-Usmar V-M, Rickwood D, Wilson MT (eds) Mitochondria — a practical approach. IRL Press Oxford, Oxford, pp 1–16

    Google Scholar 

  • Scholte HR, Pareira RR, Busch HFM et al. (1989) Carnitine deficiency, mitochondrial dysfunction and the heart. Identical defect of oxidative phosphorylation in muscle mitochondria in cardiomyopathy due to carnitine loss and in Duchenne muscular dystrophy. Wiener Klin Wochen-schr 101 (1):12–17

    CAS  Google Scholar 

  • Schotland DL, Bonilla E, Wakayama Y (1981) Freeze fracture studies of muscle plasma membrane in human muscular dystrophy. Acta Neuro-pathol 54:189

    Article  CAS  Google Scholar 

  • Sierakowski B, Elmadfa I (1987) Wie verhalten sich Retinol und Tocophe-rolkonzentrationen im Plasma von Probanden während parenteraler Verabreichung verschiedener Fettemulsionen. Akt Ernähr Med 12:93–96

    CAS  Google Scholar 

  • Sugita H, Arahata K, Takamitzu M et al. (1992) Expression of C-terminal domaine of dystrophin in Duchenne muscular dystrophy and mdx mouse. In: Kakulas B, McHowell J, Roses AD (eds) Duchenne muscular dystrophy: Animal models and genetic manipulation. Raven Press, New York, pp 67–73

    Google Scholar 

  • Tanabe Y, Esaki K, Nomura T (1986) Skeletal muscle pathology in X-chro-mosome linked muscular dystrophy (mdx-mouse). Acta Neuropathol (Berl) 69:91–95

    Article  CAS  Google Scholar 

  • Terjung RL, Hood DA (1986) Biochemical adaptations in skeletal muscle induced by exercise training. In: Layman DK (ed) Nutrition and aerobic exercise, ACS Symposium Series, The American Chemical Society, Washington DC, pp 8–26

    Chapter  Google Scholar 

  • Wieland OH, Deufel T, Paetzke-Brunner J (1985) Free and esterified Carnitine: Colorimetric method. In: Bergmeyer HU (ed) Methods of enzymatic analysis, 3rd edn, vol. III. VCH Verlagsgesellschaft Weinheim, Deer-field Beach (Florida), pp 481–488

    Google Scholar 

  • Wong S, Knight J, Hopfer S et al. (1987) Lipoperoxide in palsma as measured by liquid-chromatographic separation of malondialdehyde thiobarbitu-ric acid adduct. Clin Chem 33:214–220

    PubMed  CAS  Google Scholar 

  • Zatz M, Rapaport D, Vainhof M et al. (1991) Serum creatine-kinase (CK) and pyruvate-kinase (PK) activities in Duchenne (DMD) als compared with Becker (BMD) muscular dystrophy. J Neurol Sci 102:190–196

    Article  PubMed  CAS  Google Scholar 

  • Zöllner N, Kirsch K (1962) Über die quantitative Bestimmung von Lipoiden (Mikromethode) mittels der vielen natürlichen Lipoiden (allen bekannten Plasmalipoiden) gemeinsamen Sulfophosphovanillin-Reaktion. Z ges exp Med 135:545–561

    Article  Google Scholar 

Download references

Authors
  1. V. Faist
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. I. Elmadfa
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Zentrum der Kinderheilkunde, Klinikum der Universität Frankfurt, Theodor-Stern-Kai 7, 60559, Frankfurt/Main, Deutschland

    Hansjosef Böhles

Rights and permissions

Reprints and permissions

Copyright information

© 1995 Springer-Verlag Berlin Heidelberg

About this paper

Cite this paper

Faist, V., Elmadfa, I. (1995). Einfluß der mitochondrialen Radikalproduktion des Skelettmuskels auf die Pathogenese der Duchenne-Muskeldystrophie (mdx-Maus Modell) unter Berücksichtigung der Auswirkungen einer submaximalen physischen Belastung. In: Böhles, H. (eds) Oxidativer Stress in der Kinderheilkunde. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-79748-4_14

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-79748-4_14

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-59256-3

  • Online ISBN: 978-3-642-79748-4

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation