Beta APP gene transfer into cultured human muscle induces inclusion-body myositis aspects.
Direct transfer of the beta-amyloid precursor protein (beta APP) gene into cultured normal human muscle, using recombinant adenovirus vector, was sufficient to induce several of the typical light microscopic, electron microscopic (EM), and EM-immunochemical aspects of the inclusion-body myositis (IBM) phenotype, including congophilia, clusters of amyloid-beta-positive 6-10 nm filaments, and 15-21 nm tubulofilamentous inclusions in the nuclei. Our results suggest that excessive production of intracellular beta APP may play an important role in the pathogenic cascade leading to the IBM phenotype.
Askanas
V
V
Department of Neurology, University of Southern California, School of Medicine, Good Samaritan Hospital, Los Angeles 90017-1912, USA.
McFerrin
J
J
Alvarez
R B
RB
Baqué
S
S
Engel
W K
WK
D016564:Amyloid beta-Protein Precursor / Q000235:genetics*; D002478:Cells, Cultured; D018014:Gene Transfer Techniques; D006801:Humans; D008854:Microscopy, Electron; D018485:Muscle Fibers, Skeletal / Q000187:drug effects / Q000648:ultrastructure*; D009220:Myositis / Q000139:chemically induced*
D016564:Amyloid beta-Protein Precursor