Laura Campisi, MS, PhD
Washington University in St. Louis (WU)
Amyotrophic Lateral Sclerosis (ALS) is an incurable, heterogeneous neurodegenerative disorder that affects motor neurons in the brain and spinal cord, causing progressive loss of voluntary muscle control. Recently, we made the unexpected discovery that ALS4, a rare, slowly progressive and juvenile form of ALS, has a strong immune component, as transplantation of wild-type (WT) hematopoietic cells in ALS mutants halts disease manifestation. Strikingly, we found that increased frequencies of activated and clonally expanded CD8 T cells in the peripheral blood and in the CNS of ALS4 mice and patients are associated with the disease. We are interested in understanding whether altered T cell functions contribute to the neurodegenerative process and/or mirror disease progression in several forms of ALS.