New perspectives of treatment could be opened by a recent discovery of Mount Sinai Hospital: until now, the focus had been on the nervous system. The knot is figuring out whether neurodegeneration is caused or aggravated by an immune dysfunction
New prospects for treatment could be opened by a recent discovery regarding ALS (amyotrophic lateral sclerosis): researchers at Mount Sinai Hospital in New York have highlighted the unexpected role of the immune system in this disabling disease.
Research
The analysis, published on 22 June on Nature
, reported immune and nervous system dysfunctions in animal models and patients with ALS4, a juvenile and slowly progressive form of ALS caused by mutations in the SETX gene. Up to now, interventions have focused on the central nervous system. We learned that mutations in SETX must be expressed in both the nervous and immune systems to generate motor impairment in mice and that adaptive immune system dysfunction characterizes ALS4 in mice and humans, says Laura Campisi, assistant professor of Microbiology. co-lead author of the study with Ivan Marazzi, Associate Professor of Microbiology.
The prospects
Further evidence of immune system involvement was found in the high concentration of CD8 T cells – which are usually involved in the destruction of pathogen-harboring tumors and body cells – in the spinal cord and peripheral blood of ALS4 mice and patients. . Those increased populations of CD8 T cells, expression of the immune system, are correlated with the progression of ALS4 disease. The knot is figuring out whether neurodegeneration is caused or aggravated by an immune dysfunction. The discovery that peculiar immune signatures distinguish different forms of ALS could be significant for designing treatments tailored to specific patient subgroups. An added benefit is that ALS4-linked dysfunctional CD8 T cells can be detected in peripheral blood, which is readily accessible as compared to CSF, which requires an invasive procedure for harvesting.
The illness
ALS is characterized by the progressive death of motor neurons, which has a serious impact on the functional capacity of patients in most motor functions up to involuntary ones (such as breathing). Amyotrophic lateral sclerosis-4 (ALS4) characterized by juvenile onset and slow progression 3. Patients with ALS4 exhibit motor difficulties by the time they are in their thirties, and most require devices to help them walk in their fifties. There is no treatment or cure for ALS. In Italy alone, about 6,000 people are affected.
June 23, 2022 (change June 23, 2022 | 19:15)
© REPRODUCTION RESERVED
#ALS #role #immune #system #crippling #disease #study