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E-GEOD-34896 - Muscle specific proteasome deficient mice

Status
Released on 30 September 2014, last updated on 5 October 2014
Organism
Mus musculus
Samples (6)
Array (1)
Protocols (5)
Description
The ubiquitin-proteasome and autophagy-lysosome pathways are the two major routes for protein and organelle clearance. In skeletal muscle, both systems’ excessive activation induces severe muscle loss. Although altered proteasomal function has been observed in various myopathies, the specific role of proteasomal activity in skeletal muscle has not been determined by loss-of-function approaches. Here, we report that muscle-specific deletion of a crucial proteasomal gene, Rpt3, resulted in profound muscle atrophy and decrease in force. Rpt3 null muscles showed reduced proteasomal activity in early age, accumulation of basophilic structure, disorganization of sarcomere, and formation of vacuoles and concentric membranous structures in electronmicroscope. We also observed accumulation of ubiquitin, p62, LC3, TDP43, FUS and VCP proteins. Proteasomal activity is important to preserve muscle mass and to maintain myofiber integrity. Our results suggest that inhibition/alteration of proteasomal activity can contribute to myofiber degeneration and weakness in muscle disorders, such as inclusion body myositis, characterized by accumulation of abnormal inclusions. Tibialis anterior muscles from Rpt3 null and control mice. each 3 mice.
Experiment type
transcription profiling by array 
Contacts
Hitoshi Warita, Maki Tateyama, Masaaki Kato, Masashi Aoki, Naoki Suzuki, Risa Ando, Rumiko Izumi, Ryoichi Nagatomi, Ryosuke Takahashi, Yasuo Kitajima, Yoshihiro Tashiro
MIAME
PlatformsProtocolsVariablesProcessedRaw
Files
Investigation descriptionE-GEOD-34896.idf.txt
Sample and data relationshipE-GEOD-34896.sdrf.txt
Raw data (1)E-GEOD-34896.raw.1.zip
Processed data (1)E-GEOD-34896.processed.1.zip
Array designA-GEHB-3.adf.txt
Links