Proteomics Analysis of Lymphoblastoid Cell lines from Patients with Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) consists in the progressive degeneration of motor neurons, caused by mechanisms that are poorly understood and for which there is no cure. Some of the cellular perturbations associated with ALS can be detected in peripheral cells, including lym-phocytes from blood. A related cell system that is very suitable for research consists in human lymphoblastoid cell lines (LCLs), which are immortalized lymphocytes. LCLs that can be easily expanded in culture and can be maintained for long periods of time as stable cultures. We inves-tigated, on a small set of LCLs, if proteomics analysis by liquid chromatography followed by tandem mass spectrometry reveals proteins that are differentially present in ALS versus healthy controls. We found that individual proteins and cellular and molecular pathways in which these proteins participate, are detected as differentially present in the ALS samples. Some of these proteins and pathways are already known to be perturbed in ALS, while others are new and present interest for further investigations. These observations suggest that more detailed pro-teomics analysis of LCLs, using a larger number of samples, represents a promising approach for investigating ALS mechanisms and to search for therapeutic agents.