DIAGALS: Relation Between Tar DNA Binding Protein(TDP)-43 et Nrf-2 in ALS: a Track to Improve Diagnosis and Prognosis of the Disease

Presence of TDP-43 aggregates in PBMC,PBMC accompanied by a protein expression profile under Nrf-2 control

In response to oxidative stress, cells activate the Nrf-2 pathway, which induces translation
 of its target genes and corresponding proteins involved in the antioxidant response. This
 explains the interest in the Nrf-2 pathway in the pathophysiology of Amyotrophic lateral
 sclerosis (ALS), supported by the results of several studies and the modulatory effect of
 TDP-43 on the Nrf-2 pathway. Since both TDP-43 and Nrf-2 proteins are present in the
 peripheral blood mononuclear cells (PBMC) of ALS patients and may be correlated with disease
 progression, the investigators wish to explore their relationship and their application in
 the clinic as potential blood biomarkers for ALS.