Key result
BetaAPP overexpression completely inhibits innervation in cultured muscle fibers, preventing contraction and neuromuscular junctions.
Why the study?
Abnormal accumulation of betaAPP may be responsible for denervation of muscle fibers in hereditary and sporadic inclusion-body myopathies.
Overexpression of betaAPP in cultured muscle fibers inhibits their innervation, suggesting a 'myogenous-dysinnervation' mechanism in inclusion-body myositis.
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Should not change clinical practice in inclusion-body myositis; hypothesis-generating for betaAPP-mediated dysinnervation.
McFerrin et al. (1998) studied Hereditary inclusion-body myopathy (h-IBM) and sporadic inclusion-body myositis (s-IBM) (n=21). betaAPP overexpression vs. Normal cultured muscle fibers without betaAPP overexpression was evaluated on Muscle fiber innervation (contraction, cross-striation, and presence of neuromuscular junctions with AChRs and AChE). Overexpression of betaAPP in cultured muscle fibers completely inhibited their innervation, resulting in an absence of contraction, cross-striation, and neuromuscular junctions compared to controls.