title: Ultrastructural features of skeletal muscle in adult and aging Ts65Dn mice, a murine model of Down syndrome.
abstract: Patients with Down syndrome (DS) suffer from muscle hypotonia and an altered motor coordination whose basic mechanisms are still largely unknown. Interestingly, they show muscle weakness like healthy aged subjects, suggesting possible similarity with sarcopenia: to test this hypothesis, the Ts65Dn mouse, a suitable animal model of DS, was employed. The fine structure of skeletal fibres of the quadriceps femoris muscle was analysed in adult (12 months) and aging (19 months) animals and their age-matched euploid controls by combining morphometry and immunocytochemistry at transmission electron microscopy. Results demonstrated structural alterations of mitochondria and myonuclei reminiscent of those observed in age-related sarcopenia, supporting the hypothesis that trisomy leads to an early aging of skeletal muscle consistent with the multi-systemic premature aging typical of DS.
authors.affiliation: Department of Neurological and Movement Sciences, Anatomy and Histology Section, University of Verona, Italy.
authors.forename: Barbara
authors.initials: B
authors.lastname: Cisterna
authors.affiliation: Department of Neurological and Movement Sciences, Anatomy and Histology Section, University of Verona, Italy.
authors.forename: Manuela
authors.initials: M
authors.lastname: Costanzo
authors.affiliation: Department of Biology and Biotechnology, Laboratory of Cell Biology and Neurology, University of Pavia, Italy.
authors.forename: Elda
authors.initials: E
authors.lastname: Scherini
authors.affiliation: Department of Neurological and Movement Sciences, Anatomy and Histology Section, University of Verona, Italy.
authors.forename: Carlo
authors.initials: C
authors.lastname: Zancanaro
authors.affiliation: Department of Neurological and Movement Sciences, Anatomy and Histology Section, University of Verona, Italy.
authors.forename: Manuela
authors.initials: M
authors.lastname: Malatesta
keywords: Down syndrome; Ts65Dn mouse; aging; cell nucleus; mitochondria; skeletal muscle