Article

Myosin ATPase inhibition fails to rescue the metabolically dysregulated proteome of nebulin-deficient muscle

2024-05-10

Abstract excerpt

Nemaline myopathy (NM) is a genetic muscle disease, primarily caused by mutations in the NEB gene ( NEB- NM) and with muscle myosin dysfunction as a major molecular pathogenic mechanism. Recently, we have observed that the myosin biochemical super-relaxed state was significantly impaired in NEB -NM, inducing an aberrant increase in ATP consumption and remodelling of the energy proteome in diseased muscle fibres...

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Literature Corpus work: 6ff778f8-a151-5a29-b666-30c6515636f6
DOI: 10.1101/2024.05.07.592906

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