The neurobiology of amyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis is a degenerative disease affecting the motor neurons. In spite of our growing insights into its biology, it remains a lethal condition. The identification of the cause of several of the familial forms of ALS allowed generation of models to study this disease both in vitro and in vivo. Here, we summarize what is known about the pathogenic mechanisms of ALS induced by hereditary mutations, and attempt to identify the relevance of these findings for understanding the pathogenic mechanisms of the sporadic form of this disease.
Bento-Abreu
André
A
Laboratory for Neurobiology, Experimental Neurology, K.U.Leuven, Herestraat, 3000 Leuven, Belgium. andre.bentoabreu@vib-kuleuven.be
Van Damme
Philip
P
Van Den Bosch
Ludo
L
Robberecht
Wim
W
D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D000818:Animals; D004268:DNA-Binding Proteins / Q000235:genetics / Q000378:metabolism; D004195:Disease Models, Animal; D055106:Genome-Wide Association Study; D006801:Humans; D009154:Mutation; D009446:Neurobiology; D013482:Superoxide Dismutase / Q000235:genetics / Q000378:metabolism; D000072105:Superoxide Dismutase-1
D004268:DNA-Binding Proteins; C000606290:SOD1 protein, human; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1