Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease characterized by the loss of upper and lower motoneurons, eventually culminating in respiratory failure and death. Great insight as to what causes ALS has resulted from discovery of more than 20 gene disease-causing mutations. Clinical heterogeneity cannot be explained by altered gene expression alone, and accumulating evidence implicates neuroinflammation in modifying clinical phenotypes. In ALS neurons do not die alone; neuronal injury is non-cell-autonomous and depends on a well-orchestrated dialogue involving glia, T cells and motor neurons, mediating neuronal viability and neuronal injury. This neuroinflammatory response differs in different ALS patients: patients progressing rapidly have decreased regulatory T lymphocytic responses, while patients progressing slowly had normal to increased levels of Treg and FoxP3 expression. Therapeutic efforts targeting neuroinflammation have the potential of changing an acute rapidly progressive disease into a more slowly progressing disease and a more sustainable quality of life.
- Amyotrophic lateral sclerosis
- Diverse clinical phenotypes
- Regulatory T lymphocytes
ASJC Scopus subject areas
- Immunology and Microbiology(all)
- Pharmacology, Toxicology and Pharmaceutics(all)