Prognostic features of LGMD presenting as symptomatic or paucisymptomatic hyperCKemia

G.P.199

Our group has recently derived skeletal muscle from dermis-derived cells, by using an extracellular matrix that recreates the myogenic niche. After one week of differentiation, we observed isolated, twitching myotubes followed by spontaneous contractions of the entire tissue-engineered muscle construct. In vitro engineered myofibers expressed canonical markers, ultrastructure and electrophysiological characteristics of skeletal muscle. Interestingly, after one-month engineered muscle constructs showed progressive degradation of the myofibers concomitant with fatty infiltration, paralleling the natural course of muscular degeneration. However, we do not yet know how dermis-resident precursor…