Détail de la notice
Titre du Document
Motor neurones in culture as a model to study ALS
Auteur(s)
SILANI V. ; BRAGA M. ; CIAMMOLA A. ; ...
Résumé
Defining the basis of the selective cell vulnerability of motor neurones (MN) represents the key issue in amyotrophic lateral sclerosis (ALS), and tissue culture models are the ideal system for the identification of the MN specific features at the single cell level. Neurone-astrocyte metabolic interactions, which have a critical role in MN through glutamatergic toxicity, have been mostly defined in vitro. Ca++ metabolism, which appears to play a critical role in inducing MN loss in ALS, has been successfully studied using in vitro cell models. Furthermore, primary cultures demonstrated that apoptotic or necrotic death of neurones after injury depends upon the cell energetic status. Superoxide dismutase-1 (SOD-1) mutations were successfully expressed in cultured rodent MNs, providing a critical assay to sequence the molecular processes responsible for MN degeneration due to the identified genetic defect. The recent identification of genes that separate humans from apes further increases the value of the human in vitro models to better understand specific human cellular properties. Purified human MNs and astrocytes can today be obtained from the human embryonic spinal cord anterior horns. Interactions at the single cell level can be dissected using the cDNA amplification techniques. The effects of molecules affecting MN survival, neurite extension, and metabolism can easily be defined in vitro, gaining a critical mass of information of immediate clinical application in th
Editeur
Springer
Type du document
Conférence : Molecular Issues in Motor Neuron Diseases. Symposium, Nice, FRA, 1998-06-11
Identifiant
PMID : 10795884 ISSN : 0939-1517
Source
Journal of neurology. Supplement A. 2000, vol. 247, n° 1, pp. 28-36 [bibl. : 61 ref.]
Langue
Anglais
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