Annual review of pathology

Problems with Cell Cleanup in Nerve and Muscle Diseases: From Genetic Causes to Treatment Progress

Updated

Abstract

Severe autophagy defects are reported in numerous congenital disorders of striated muscle and inherited neuropathies.

  • Genetic mutations linked to lower motor neuron diseases, skeletal muscle dystrophies, and (cardio)myopathies may disrupt autophagy pathways.
  • The degradation of damaged proteins and organelles is essential for maintaining cell function under stress.
  • Identifying defective steps in the autophagy pathway could lead to new pharmacological targets for treating related diseases.
  • Current limitations exist in developing drugs that modulate autophagy effectively.
  • Novel technologies may support advancements in creating better autophagy modulators for rare diseases.

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