Portal de Periódicos da CAPES

The neurobiology of amyotrophic lateral sclerosis

2010; Wiley; Volume: 31; Issue: 12 Linguagem: Inglês

10.1111/j.1460-9568.2010.07260.x

1460-9568

André Bento‐Abreu, Philip Van Damme, Ludo Van Den Bosch, Wim Robberecht,

Neurological diseases and metabolism

Abstract 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.