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B37 Investigation Of Viability And Response To Inflammatory Stimuli In Cultured Human Myotubes Derived From Patients With Huntington’s Disease
  1. JA Parker1,
  2. S Haider1,
  3. JRC Miller1,
  4. S Brown1,
  5. N Robertson1,
  6. M Lewis1,
  7. E Sillery1,
  8. V Nowak1,
  9. H Sethi1,
  10. M Björkqvist2,
  11. M Orth3,
  12. SJ Tabrizi1
  1. 1UCL Institute of Nuerology, Queen Square, London WC1N 3BG, UK
  2. 2Lund University, Sölvegatan 17, 221 84 Lund, Sweden
  3. 3University of Ulm, Oberer Eselsberg 45/1, 89081 Ulm, Germany

Abstract

Background Muscle atrophy has been well documented in patients with HD and yet its cause is unknown. The presence of a low grade immune response in HD has been implicated in muscle wasting. Myoblasts and myotubes within skeletal muscle are known not only to respond to inflammatory cytokines including IL-6 and TNFα, but also release cytokines. The presence of mutant HTT within the myoblasts and myotubes of HD patients may affect the release of cytokines from these cells as has previously been observed in HD monocytes and macrophages.

Aims To assess the viability and response to inflammatory stimuli in human HD myotubes in vitro.

Methods As part of the Multi-Tissue Molecular signatures in HD project (MTM-HD) we have collected muscle biopsies from early stage HD patients and matched controls. Myoblast cultures have been established from these biopsies and differentiated into myotubes. Differentiation has been assessed using immunofluorescent staining for myoblast and myotube markers. Release of IL-6 has been measured using an electrochemiluminescent assay.

Results The myoblast content and differentiation potential of these cultures from control and early HD patients have been determined and compared. The release of IL-6 in response to lipopolysaccharide (LPS), TNFα and IL-6 by myoblasts and myotubes from control and early HD patients was measured. As HD is an age-related disease, the effects of ageing myotubes in vitroon their viability and response to LPS have also been assessed.

Conclusions Here data is presented comparing the differentiation of HD and control myoblasts, their survival in culture and response to inflammatory stimuli.

KeyWords
  • Muscle
  • Inflammation
  • myotube

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