Article
Focal caspase activation underlies the endplate myopathy in slow-channel syndrome.
Annals of neurology - 1 Mar 2004
Vohra Bhupinder P S, Groshong Jason S, Maselli Ricardo A, Verity M Anthony, Wollmann Robert L, Gomez Christopher M
Abstract excerpt
Slow-channel syndrome (SCS) is a progressive neuromuscular disorder caused by abnormal gating of mutant acetylcholine receptors (AChRs) in the neuromuscular junction (NMJ). The pathological hallmark is selective degeneration of the NMJ termed endplate myopathy. Endplate myopathy consists of a combination of ultrastructural abnormalities, including degenerating subsynaptic nuclei, mitochondria, and postsynaptic...
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