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C17 Increased mitochondrial number, cellular stress and glycogen accumulation in heart of minipig model transgenic for N-terminal part of human mutated huntingtin
  1. Marie Rodinova1,
  2. Nina Ondruskova1,
  3. Zaneta Dosoudilova1,
  4. Stefan Juhas2,
  5. Jana Juhasova2,
  6. Zdenka Ellederova2,
  7. Jan Motlik2,
  8. Hana Hansikova1
  1. 1Department of Paediatrics and Adolescent Medicine, First Faculty of Medicine, Charles University in Prague and General University Hospital in Prague, Czech Republic
  2. 2Laboratory of Cell Regeneration and Cell Plasticity, Institute of Animal Physiology and Genetics AS CR, v.v.i. Libechov, Czech Republic


Background Minipig model transgenic for N-terminal part of human mutated huntingtin (TgHD) created in Libechov allows us to study the development of Huntington’s disease (HD) in the long-term context.

Aim To monitor mitochondrial structure and metabolism in high energy demand tissues from TgHD and WT minipigs.

Material and methods Ultrastructure was analysed by transmission electron microscopy, oxidative phosphorylation system (OXPHOS) complexes activity and content were measured by spectrophotometric and immunoelectrophoretic methods in heart, muscle and brain of 36 and 48 month (M) old animals. Furthermore, polarographic measurement of respiration and analysis of capacity of mitochondrial energy generation system (MEGS) were performed in muscle.

Results Increased number of mitochondria, increased fibrosis, glycogen accumulation and slightly decreased levels of selected OXPHOS subunits were detected in 48 M old TgHD heart. Decreased respiration and altered capacity of MEGS and levels of OXPHOS complexes I, III and IV were observed in muscle. Slight alterations of ultrastructure in basal ganglia and altered activities of OXPHOS complexes in frontal cortex were detected in 48 M old TgHD brain.

Conclusion Our results contribute to understanding of physiological consequences of the earliest changes – preclinical signs in different tissues manifest in HD.

Supported by Czech-Norwegian Financial Mechanism 2009–2014 and MSMT under project “HUNTINGTON” 7F14308, COST LD15099 (MSMT) and NPU LO1609 (MSMT)

  • Mitochondria
  • heart ultrastructure
  • basal ganglia

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