NR AXRJ
AU Liang,J.; Huang,S.; Li,X.; Zheng,M.; Wang,M.; Hays,A.P.; Gambetti,P.; Booth,S.; Kong,Q.
TI PrP-Mediated Myopathy in an Inducible Transgenic Mouse Model
QU International Conference - Prion 2007 (26.-28.9.2007) Edinburgh International Conference Centre, Edinburgh, Scotland, UK - Book of Abstracts: Pathology and Pathogenesis P03.189
IA http://www.prion2007.com/pdf/Prion Book of Abstracts.pdf
PT Konferenz-Poster
AB The prion protein (PrP) level in muscles has been reported to be elevated in patients with inclusion-body myositis, polymyositis, dermatomyositis and neurogenic muscle atrophy, but it was not clear whether the elevated PrP accumulation in the muscles is sufficient to cause muscle diseases. We have generated transgenic mice with musclespecific expression of PrP under extremely tight regulation by doxycycline, and demonstrated that over-expression of wild type PrP in skeletal muscles is sufficient to cause a primary myopathy with no signs of peripheral neuropathy, possibly due to accumulation of a cytotoxic truncated form of PrP and/or PrP aggregation. Here we will report our progress in investigating the molecular mechanism of PrP-mediated myopathy, especially on the regulation of several critical genes in association with the PrP-mediated pathogenic process in skeletal muscles. One of the genes that are down-regulated in affected skeletal muscles is transcription factor MEF2c, which may underline the central nucleus localization observed in many muscle fibers. The potential role of truncated PrP fragments in natural muscle diseases will also be explored.
AD J. Liang, S. Huang, Xinyi Li, M. Zheng, M. Wang, P. Gambetti, Q. Kong, Case Western Reserve University, Pathology, USA; A.P. Hays, Columbia University Medical Center, Pathology, USA; S. Booth, National Microbiology Laboratory, Division of Host Genetics and Prion Diseases, Canada
SP englisch
PO Schottland
EA pdf-Datei und Poster (Posterautoren ergänzt um P. Wang und B. Chakraborty)