Skip to Main content Skip to Navigation
Journal articles

[Amyotrophic lateral sclerosis: role of energy deficiency in neuromuscular junction dismantlement.]

Abstract : Amyotrophic lateral sclerosis (ALS) is the most frequent adult onset motor neuron disorder. A subset of ALS cases is linked to mutations in the copper/zinc superoxide dismutase (sod1) gene and detailed phenotypic analysis of transgenic mice overexpressing mutant forms of SOD1 (mSOD1) allowed a better understanding of the pathophysiological mechanisms leading to motor neuron death. The promising results obtained in these animal models however poorly translated into conclusive clinical trials. In this review, we summarize the main pathological mechanisms at work in mSOD1 mice. In particular, recent results showed that the key pathological event was the destruction of the neuromuscular junction rather than motor neuron death. Neuromuscular junction dismantlement is likely the result of a chronic energy deficiency at the level of the whole organism. These results, along with a comparative analysis between the phenotype of mSOD1 mice and ALS patients, suggest new therapeutic strategies and show the interests but also the limits of the animal models. double dagger.
Document type :
Journal articles
Complete list of metadatas

Cited literature [40 references]  Display  Hide  Download

https://www.hal.inserm.fr/inserm-00351072
Contributor : Luc Dupuis <>
Submitted on : Thursday, January 8, 2009 - 1:54:15 PM
Last modification on : Thursday, April 23, 2020 - 2:26:29 PM
Long-term archiving on: : Tuesday, June 8, 2010 - 7:02:36 PM

File

dupuisloeffler.pdf
Files produced by the author(s)

Identifiers

  • HAL Id : inserm-00351072, version 1
  • PUBMED : 19116118

Collections

Citation

Luc Dupuis, Jean-Philippe Loeffler. [Amyotrophic lateral sclerosis: role of energy deficiency in neuromuscular junction dismantlement.]. médecine/sciences, EDP Sciences, 2008, 24 (12), pp.1077-82. ⟨inserm-00351072⟩

Share

Metrics

Record views

266

Files downloads

320