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Year : 2012  |  Volume : 60  |  Issue : 5  |  Page : 510--511

Cytoskeletal and extracellular matrix alterations in limb girdle muscular dystrophy 2I muscle fibers

1 Institute of Molecular Genetic-CNR, Rizzoli Orthopedic Institute, Bologna, Italy
2 Department of Rizzoli-Sicily, Rizzoli Orthopedic Institute, Bologna, Italy
3 Department of Rizzoli-Sicily, Physical Medicine and Rehabilitation, Rizzoli Orthopedic Institute, Bagheria, Italy

Correspondence Address:
Luciano Merlini
Physical Medicine and Rehabilitation, Department of Rizzoli-Sicily, Rizzoli Orthopedic Institute, SS 113 km 246, 90011 Bagheria
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Source of Support: Grant. No. GUP11007 Telethon Italy, Conflict of Interest: None

DOI: 10.4103/0028-3886.103200

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In this detailed muscle biopsy study of a patient with molecularly confirmed diagnosis of limb-girdle muscular dystrophy 2I (LGMD2I) we show some new data, that is the presence of altered expression pattern of costamere components as integrin α7B and integrin β1D associated with vinculin costameric derangement and basal lamina ultrastructural abnormalities as detachments and discontinuities suggesting that different cellular compartments are involved in LGMD2I and the altered basement membrane-plasmalemma-cytoskeleton binding can underlie muscle degeneration.


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Online since 20th March '04
Published by Wolters Kluwer - Medknow