Population
Transgenic mouse model expressing an autosomal dominant mutant of alpha-tropomyosin to study nemaline myopathy
Design
Preclinical
Follow-up
Up to 5-6 months of age
Authors
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Highlights fiber atrophy as late weakness driver in nemaline myopathy models; leaves open human translation and therapeutic targeting.
In a mouse model of nemaline myopathy, late-onset muscle weakness correlates with an age-related decrease in fiber diameter, suggesting that early weakness is prevented by compensatory hypertrophy of fast, glycolytic fibers.
Mark Corbett (2001) studied this question.
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