Metabolomics,Unknown,Transcriptomics,Genomics,Proteomics

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Transcription profiling of skeletal and cardiac muscles in dysferlin-deficient SJL/J mice and wild type mice to understand the protective mechanisms against dysferlin deficiency in cardiac muscles.

ABSTRACT: Dysferlin is expressed in skeletal and cardiac muscle. However, dysferlin deficiency, namely limb girdle muscular dystrophy 2B (LGMD2B) and Myoshi myopathy, results in skeletal muscle weakness and spares the heart. This dichotomy could be caused by differential regulation of protective mechanisms. Therefore, we compared intraindividual mRNA expression profiles between cardiac and skeletal muscle in dysferlin-deficient SJL/J mice and normal C57BL/6 mice. Experiment Overall Design: 20 chips were analyzed. They represent 4 groups of 5 replicates each. Experiment Overall Design: The 4 groups are cardiac (LV) and skeletal muscle of normal and dysferlin deficient mice. Experiment Overall Design: Tissues from normal mice are the controls in comparison to tissues of dysferlin deficient mice.

ORGANISM(S): Mus musculus

SUBMITTER: Oliver Hummel 

PROVIDER: E-GEOD-2507 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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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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