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Titre du document / Document title

Motor neurones in culture as a model to study ALS

Auteur(s) / Author(s)

SILANI V. (1) ; BRAGA M. (1) ; CIAMMOLA A. (1) ; CARDIN V. (1) ; SCARLATO G. (1) ;

Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)

(1) The Institute of Neurology, University of Milan Medical School - I.R.C.C.S. Maggiore Hospital, Via F. Sforza 35, 20122 Milan, ITALIE

Résumé / Abstract

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 the treatment of patients affected by ALS. Understanding the properties of human MNs in vitro represents today a significant and critical tool that can easily be reached after extension of the available knowledge from non-primate to human research. Human MN culture studies can greatly contribute to identifying the primitive critical cellular events responsible for the MN degeneration observed in ALS and to gaining crucial information on new therapeutical agents.

Revue / Journal Title

Journal of neurology. Supplement    ISSN  0939-1517 

Source / Source

Congrès
Molecular Issues in Motor Neuron Diseases. Symposium, Nice , FRANCE (11/06/1998)
2000, vol. 247, no 1 (44 p.)  (61 ref.), [Notes: Satellite symposium of the European Neurological Society-8th Meeting], pp. 28-36

Langue / Language

Anglais

Editeur / Publisher

Springer, Berlin, ALLEMAGNE  (1985) (Revue)

Mots-clés anglais / English Keywords

Amyotrophic lateral sclerosis

;

Cell culture

;

Motor neuron

;

Astrocyte

;

Calcium ion

;

Glutamate

;

Pathogenesis

;

Cell cell interaction

;

Metabolism

;

Human

;

Nervous system diseases

;

Central nervous system disease

;

Spinal cord disease

;

Degenerative disease

;

Mots-clés français / French Keywords

Sclérose latérale amyotrophique

;

Culture cellulaire

;

Neurone moteur

;

Astrocyte

;

Calcium ion

;

Glutamate

;

Pathogénie

;

Interaction cellulaire

;

Métabolisme

;

Homme

;

Système nerveux pathologie

;

Système nerveux central pathologie

;

Moelle épinière pathologie

;

Maladie dégénérative

;

Mots-clés espagnols / Spanish Keywords

Esclerosis lateral amiotrófica

;

Cultivo celular

;

Neurona motora

;

Astrocito

;

Calcio ión

;

Glutamato

;

Patogenia

;

Interacción celular

;

Metabolismo

;

Hombre

;

Sistema nervioso patología

;

Sistema nervosio central patología

;

Médula espinal patología

;

Enfermedad degenerativa

;

Localisation / Location

INIST-CNRS, Cote INIST : 4826 S, 35400008703960.0040

Nº notice refdoc (ud4) : 1353617



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