Dataset Information


Mus musculus

ABSTRACT: Cardiac and skeletal muscle gene expression profiles in dysferlin deficient mice

ORGANISM(S): Mus musculus  



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Increased susceptibility to complement attack due to down-regulation of decay-accelerating factor/CD55 in dysferlin-deficient muscular dystrophy.

Wenzel Katrin K   Zabojszcza Joanna J   Carl Miriam M   Taubert Semjon S   Lass Antje A   Harris Claire L CL   Ho Mengfatt M   Schulz Herbert H   Hummel Oliver O   Hubner Norbert N   Osterziel Karl Josef KJ   Spuler Simone S  

Journal of immunology (Baltimore, Md. : 1950) 20051101 9

Dysferlin is expressed in skeletal and cardiac muscles. However, dysferlin deficiency results in skeletal muscle weakness, but spares the heart. We compared intraindividual mRNA expression profiles of cardiac and skeletal muscle in dysferlin-deficient SJL/J mice and found down-regulation of the complement inhibitor, decay-accelerating factor/CD55, in skeletal muscle only. This finding was confirmed on mRNA and protein levels in two additional dysferlin-deficient mouse strains, A/J mice and Dysf-  ...[more]

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