Impairment of caveolae formation and T-system disorganization in human muscular dystrophy with caveolin-3 deficiency.

@article{Minetti2002ImpairmentOC,
  title={Impairment of caveolae formation and T-system disorganization in human muscular dystrophy with caveolin-3 deficiency.},
  author={Carlo Minetti and Massimo Bado and Paolo Broda and Federica Sotgia and Claudio Bruno and Ferruccio Galbiati and Daniela Volont{\'e} and Giuseppe Lucania and Antonio Pavan and Eduardo Bonilla and Michael Phillip Lisanti and Giuseppe Cordone},
  journal={The American journal of pathology},
  year={2002},
  volume={160 1},
  pages={265-70}
}
Caveolin-3, a muscle specific caveolin-related protein, is the principal structural protein of caveolar membranes. We have recently identified an autosomal dominant form of limb girdle muscular dystrophy (LGMD-1C) that is due to caveolin-3 deficiency and caveolin-3 gene mutations. Here, we studied by electron microscopy, including freeze-fracture and lanthanum staining, the distribution of caveolae and the organization of the T-tubule system in caveolin-3 deficient human muscle fibers. We found… CONTINUE READING
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