Genetics of amyotrophic lateral sclerosis

Rev Neurol (Paris). 2017 May;173(5):254-262. doi: 10.1016/j.neurol.2017.03.030. Epub 2017 Apr 25.

Abstract

Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease characterized by upper and lower motor neuron damage in the bulbar and spinal territories. Although the pathophysiology of ALS is still unknown, the involvement of genetic factors is no longer a subject of debate. Familial ALS (fALS) accounts for 10-20% of cases. Since the identification of the SOD1 gene, more than 20 genes have been described, of which four can explain >50% of familial cases. This review is an update focused on major aspects of the field of ALS genetics concerning both causative and susceptibility factors.

Keywords: ALS; Association studies; Familial ALS; Genes; Genetic; SALS; Sporadic ALS; fALS.

Publication types

  • Review

MeSH terms

  • Amyotrophic Lateral Sclerosis / genetics*
  • C9orf72 Protein / genetics
  • Humans
  • Superoxide Dismutase-1 / genetics

Substances

  • C9orf72 Protein
  • C9orf72 protein, human
  • SOD1 protein, human
  • Superoxide Dismutase-1