Review of the Pathology of Muscle in Amyotrophic Lateral Sclerosis - PubMed
3 hours ago
- #Amyotrophic lateral sclerosis
- #Neuromuscular Junction
- #Muscle Pathology
- In ALS, a central event is motor nerve terminal withdrawal from muscle, with ongoing debate on whether motor neuron or muscle faults drive this.
- The review outlines skeletal muscle basics, including fiber types, motor units, and the neuromuscular synapse, focusing on human ALS pathology.
- Clinical and biomarker changes in ALS patients' muscles are described, including histopathological changes like fiber type grouping and inflammation.
- Other muscle abnormalities covered include protein misfolding, mitochondrial dysfunction, and alterations at neuromuscular junctions and satellite cells.
- Metabolic and molecular-genetic changes in ALS muscle are updated, suggesting muscle could be an additional therapy target alongside CNS-focused treatments.