Key result
DMRV mouse model exhibits age-related muscle weakness, reduced contractile power, and late-onset rimmed vacuoles.
Why the study?
The pathomechanism of muscle weakness and atrophy in distal myopathy with rimmed vacuoles (DMRV)/hereditary inclusion body myopathy (hIBM) was not fully understood.
Population
Gne(-/-)hGNED176VTg mouse model of DMRV/hIBM
Comparison
Age-related changes in muscle function and pathology
Design
Experimental study in a mouse model
Authors
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Offers DMRV mouse model for age-related myopathy studies; hypothesis-generating for human translation pending prospective validation.
Malicdan et al. (2008) studied Distal myopathy with rimmed vacuoles (DMRV) / hereditary inclusion body myopathy (hIBM). Gne(-/-)hGNED176VTg mouse model was evaluated on Overall motor performance, structure and function of isolated skeletal muscles, and muscle pathology. The DMRV mouse model exhibited age-related muscle weakness, decreased muscle mass and cross-sectional area, and reduced contractile power, with rimmed vacuoles appearing in older age.
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