title: Beta-2 microglobulin is important for disease progression in a murine model for amyotrophic lateral sclerosis.
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 (SOD1 (G93A) ) 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.
authors.affiliation: KU Leuven Laboratory of Neurobiology and Leuven Research Institute for Neuroscience and Disease (LIND) Leuven, Belgium ; VIB Vesalius Research Center, KU Leuven Leuven, Belgium ; VIB Autoimmune Genetics Laboratory, KU Leuven Leuven, Belgium ; Department of Microbiology and Immunology, University of Leuven Leuven, Belgium.
authors.forename: Kim A
authors.initials: KA
authors.lastname: Staats
authors.affiliation: VIB Autoimmune Genetics Laboratory, KU Leuven Leuven, Belgium ; Department of Microbiology and Immunology, University of Leuven Leuven, Belgium.
authors.forename: Susann
authors.initials: S
authors.lastname: Schönefeldt
authors.affiliation: VIB Autoimmune Genetics Laboratory, KU Leuven Leuven, Belgium ; Department of Microbiology and Immunology, University of Leuven Leuven, Belgium.
authors.forename: Marike
authors.initials: M
authors.lastname: Van Rillaer
authors.affiliation: KU Leuven Laboratory of Neurobiology and Leuven Research Institute for Neuroscience and Disease (LIND) Leuven, Belgium ; VIB Vesalius Research Center, KU Leuven Leuven, Belgium.
authors.forename: Annelies
authors.initials: A
authors.lastname: Van Hoecke
authors.affiliation: KU Leuven Laboratory of Neurobiology and Leuven Research Institute for Neuroscience and Disease (LIND) Leuven, Belgium ; VIB Vesalius Research Center, KU Leuven Leuven, Belgium ; Neurology, University Hospitals Leuven Leuven, Belgium.
authors.forename: Philip
authors.initials: P
authors.lastname: Van Damme
authors.affiliation: KU Leuven Laboratory of Neurobiology and Leuven Research Institute for Neuroscience and Disease (LIND) Leuven, Belgium ; VIB Vesalius Research Center, KU Leuven Leuven, Belgium ; Neurology, University Hospitals Leuven Leuven, Belgium.
authors.forename: Wim
authors.initials: W
authors.lastname: Robberecht
authors.affiliation: VIB Autoimmune Genetics Laboratory, KU Leuven Leuven, Belgium ; Department of Microbiology and Immunology, University of Leuven Leuven, Belgium.
authors.forename: Adrian
authors.initials: A
authors.lastname: Liston
authors.affiliation: KU Leuven Laboratory of Neurobiology and Leuven Research Institute for Neuroscience and Disease (LIND) Leuven, Belgium ; VIB Vesalius Research Center, KU Leuven Leuven, Belgium.
authors.forename: Ludo
authors.initials: L
authors.lastname: Van Den Bosch
keywords: amyotrophic lateral sclerosis; beta-2 microglobulin; motor neuron; motor neuron disease; neurodegeneration