AUTHOR=Staats Kim A., Schonefeldt Susann , Van Rillaer Marieke , Van Hoecke Annelies , Van Damme Philip , Robberecht Wim , Liston Adrian , Van Den Bosch Ludo TITLE=Beta-2 microglobulin is important for disease progression in a murine model for amyotrophic lateral sclerosis JOURNAL=Frontiers in Cellular Neuroscience VOLUME=7 YEAR=2013 URL=https://www.frontiersin.org/journals/cellular-neuroscience/articles/10.3389/fncel.2013.00249 DOI=10.3389/fncel.2013.00249 ISSN=1662-5102 ABSTRACT=

Beta-2 microglobulin (β2m) is an essential component of the major histocompatibility complex (MHC) class I proteins and in the nervous system β2m is predominantly expressed in motor neurons. As β2m can promote nerve regeneration, we investigated its potential role in amyotrophic lateral sclerosis (ALS) by investigating its expression level as well as the effect of genetically removing β2m on the disease process in mutant superoxide dismutase 1 (SOD1G93A) mice, a model of ALS. We observed a strong upregulation of β2m in motor neurons during the disease process and ubiquitous removal of β2m dramatically shortens the disease duration indicating that β2m plays an essential and positive role during the disease process. We hypothesize that β2m contributes to plasticity that is essential for muscle reinnervation. Absence of this plasticity will lead to faster muscle denervation and counteracting this process could be a relevant therapeutic target.