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2005 | 52 | 2 | 453-460
Article title

Ultrastructure of diaphragm from dystrophic α-sarcoglycan-null mice.

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Abstracts
EN
α-Sarcoglycan is a 50 kDa single-pass transmembrane glycoprotein exclusively expressed in striated muscle that, together with β-, γ-, and δ-sarcoglycan, forms a sub-complex at the muscle fibre cell membrane. The sarcoglycans are components of the dystrophin-associated glycoprotein (DAG) complex which forms a mechanical link between the intracellular cytoskeleton and extracellular matrix. The DAG complex function is to protect the muscle membrane from the stress of contractile activity and as a structure for the docking of signalling proteins. Genetic defects of DAG components cause muscular dystrophies. A lack or defects of α-sarcoglycan causes the severe type 2D limb girdle muscular dystrophy. α-Sarcoglycan-null (Sgca-null) mice develop progressive muscular dystrophy similar to the human disorder. This animal model was used in the present work for an ultrastructural study of diaphragm muscle. Diaphragm from Sgca-null mouse presents a clear dystrophic phenotype, with necrosis, regeneration, fibre hypertrophy and splitting, excess of collagen and fatty infiltration. Some abnormalities were also observed, such as centrally located nuclei of abnormal shape, fibres containing inclusion bodies within the contractile structure, and fibres with electron-dense material dispersed over almost the entire cell. Additionally, unusual interstitial cells of uncertain identity were detected within muscle fibres. The abnormal ultrastructure of the diaphragm from Sgca-null mice is discussed.
Publisher

Year
Volume
52
Issue
2
Pages
453-460
Physical description
Contributors
  • Nencki Institute of Experimental Biology, Warszawa, Poland
  • CNR Institute of Neuroscience, Laboratory of Muscle Biology and Physiopathology, Department of Biomedical Sciences, University of Padova, Padova, Italy
  • Nencki Institute of Experimental Biology, Warszawa, Poland
author
  • CNR Institute of Neuroscience, Laboratory of Muscle Biology and Physiopathology, Department of Biomedical Sciences, University of Padova, Padova, Italy
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Document Type
Publication order reference
Identifiers
YADDA identifier
bwmeta1.element.bwnjournal-article-abpv52i2p453kz
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