Cultured myoblasts from patients affected by myotonic dystrophy type 2 exhibit senescence-related features: ultrastructural evidence

  • M. Malatesta | manuela.malatesta@univr.it Dip. di Scienze Scienze Neurologiche, Neuropsicologiche, Morfologiche e Motorie. Sezione di Anatomia e Istologia, Italy.
  • M. Giagnacovo University of Pavia, Italy.
  • L.V. Renna University of Milano Centro per lo Studio delle Malattie Neuromuscolari – CMN, Italy.
  • R. Cardani University of Milano Centro per lo Studio delle Malattie Neuromuscolari – CMN, Italy.
  • G. Meola University of Milano, Italy.
  • C. Pellicciari University of Pavia, Italy.

Abstract

Myotonic dystrophy type 2 (DM2) is an autosomal dominant disorder caused by the expansion of the tetranucleotidic repeat (CCTG)n in the first intron of the Zinc Finger Protein-9 gene. In DM2 tissues, the expanded mutant transcripts accumulate in nuclear focal aggregates where splicing factors are sequestered, thus impairing the whole mRNA processing. Interestingly, the ultrastructural alterations in the splicing machinery observed in the myonuclei of DM2 skeletal muscles are reminiscent of the nuclear changes occurring in age-related muscle atrophy. Here, we investigated structural and functional features of satellite cell-derived myoblasts from biceps brachii, in the attempt to investigate cell senescence indices in DM2 patients by ultrastructural cytochemistry. We observed that in satellite cell-derived DM2 myoblasts, cell-senescence alterations and impairment of the pre-mRNA maturation pathways occur earlier than in myoblasts from healthy patients. This suggests that also in vivo the regeneration capability of satellite cells could be reduced in dystrophic muscles.

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Author Biographies

M. Giagnacovo, University of Pavia

Dipartimento di Biologia Animale, Laboratorio di Biologia Cellulare

L.V. Renna, University of Milano Centro per lo Studio delle Malattie Neuromuscolari – CMN

Dipartimento di Biologia molecolare e Biotecnologie

R. Cardani, University of Milano Centro per lo Studio delle Malattie Neuromuscolari – CMN

Dipartimento di Biologia molecolare e Biotecnologie

G. Meola, University of Milano

Dipartimento di Neurologia, IRCCS Policlinico San Donato

C. Pellicciari, University of Pavia

Dipartimento di Biologia Animale, Laboratorio di Biologia Cellulare

Published
2011-09-21
Section
Original Papers
Keywords:
myotonic dystrophy type 2 – DM2, myoblasts, RNA processing, electron microscopy, immunocytochemistry
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How to Cite
Malatesta, M., Giagnacovo, M., Renna, L., Cardani, R., Meola, G., & Pellicciari, C. (2011). Cultured myoblasts from patients affected by myotonic dystrophy type 2 exhibit senescence-related features: ultrastructural evidence. European Journal of Histochemistry, 55(3), e26. https://doi.org/10.4081/ejh.2011.e26