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=<p>Beta-2 microglobulin (<italic>β</italic>2<italic>m</italic>) is an essential component of the major histocompatibility complex (MHC) class I proteins and in the nervous system <italic>β</italic>2<italic>m</italic> is predominantly expressed in motor neurons. As <italic>β</italic>2<italic>m</italic> 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 <italic>β</italic>2<italic>m</italic> on the disease process in mutant superoxide dismutase 1 (<italic>SOD1</italic><sup><italic>G</italic>93<italic>A</italic></sup>) mice, a model of ALS. We observed a strong upregulation of <italic>β</italic>2<italic>m</italic> in motor neurons during the disease process and ubiquitous removal of <italic>β</italic>2<italic>m</italic> dramatically shortens the disease duration indicating that <italic>β</italic>2<italic>m</italic> plays an essential and positive role during the disease process. We hypothesize that <italic>β</italic>2<italic>m</italic> 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.</p>