Publications

Dernière publication 27/02/2008

Neuron-Specific Expression of Mutant Superoxide Dismutase Is Sufficient to Induc

Mutations in superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS), an adult-onset progressive paralytic disease characterized by...

Array
(
    [id_prestablog_news] => 148
    [id_shop] => 1
    [date] => 2008-02-27 00:00:00
    [date_modification] => 2024-02-09 14:15:11
    [langues] => ["1","2"]
    [actif] => 1
    [slide] => 0
    [url_redirect] => 
    [average_rating] => 
    [number_rating] => 
    [author_id] => 1
    [featured] => 0
    [ishero] => 0
    [prim_key] => 294
    [id_lang] => 2
    [title] => Neuron-Specific Expression of Mutant Superoxide Dismutase Is Sufficient to Induc
    [paragraph] => Neuron-Specific Expression of Mutant Superoxide Dismutase Is Sufficient to Induce Amyotrophic Lateral Sclerosis in Transgenic Mice.
    [content] => 
Authors
D. Jaarsma, E. Teuling, E. Haasdijk, C. De Zeeuw, C. Hoogenraad.

 Lab
Erasmus Medical Center, Department of Neuroscience, Rotterdam, The Netherlands.
Journal
The Journal of Neuroscience
Abstract
Mutations in superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS), an adult-onset progressive paralytic disease characterized by loss of motor neurons, and cause an ALS-like disease when expressed in mice. Recent data have suggested that motor neuron degeneration results from toxic actions of mutant SOD1 operating in both motor neurons and their neighboring glia, raising the question whether mutant SOD1 expression selectively in neurons is sufficient to induce disease. Here we show that neuronal expression of mutant SOD1 is sufficient to cause motor neuron degeneration and paralysis in transgenic mice with cytosolic dendritic ubiquitinated SOD1 aggregates as the dominant pathological feature. In addition, we show that crossing our neuron-specific mutant SOD1 mice with ubiquitously wild-type SOD1-expressing mice leads to dramatic wild-type SOD1 aggregation in oligodendroglia after the onset of neuronal degeneration. Together, our findings support a pathogenic scenario in which mutant SOD1 in neurons triggers neuronal degeneration, which in turn may facilitate aggregate formation in surrounding glial cells.
BIOSEB Instruments Used
Grip strength test (BIO-GS3)

Keywords/Topics
Sclérose Latérale Amyotrophique (SLA), ou Maladie de Charcot; Phénotypage; Système musculaire; Thématiques transversales
    [meta_description] => 
    [meta_keywords] => http://www.neuro.cjb.net/content/28/9/2075.short
    [meta_title] => 
    [link_rewrite] => neuron-specific-expression-of-mutant-superoxide-dismutase-is-sufficient-to-induce-amyotrophic-lateral-sclerosis-in-transgenic-mice-
    [actif_langue] => 1
    [read] => 932
    [count_comments] => 0
    [id] => 148
    [categories] => Array
        (
            [87] => Array
                (
                    [id_prestablog_categorie] => 87
                    [title] => Phénotypage
                    [link_rewrite] => Phenotypage
                )

            [2] => Array
                (
                    [id_prestablog_categorie] => 2
                    [title] => Publications
                    [link_rewrite] => publications
                )

            [67] => Array
                (
                    [id_prestablog_categorie] => 67
                    [title] => Sclérose Latérale Amyotrophique (SLA), ou Maladie de Charcot
                    [link_rewrite] => Sclerose-Laterale-Amyotrophique-SLA-ou-Maladie-de-Charcot
                )

            [20] => Array
                (
                    [id_prestablog_categorie] => 20
                    [title] => Système musculaire
                    [link_rewrite] => Systeme-musculaire
                )

            [24] => Array
                (
                    [id_prestablog_categorie] => 24
                    [title] => Thématiques transversales
                    [link_rewrite] => Thematiques-transversales
                )

        )

    [authors] => 
    [paragraph_crop] => Neuron-Specific Expression of Mutant Superoxide Dismutase Is Sufficient to Induce Amyotrophic [...]
    [link_for_unique] => 1
    [products_liaison] => Array
        (
            [48] => Array
                (
                    [name] => Test d'agrippement
                    [description_short] => 
Une méthode simple pour quantifier objectivement la force musculaire des rats et souris et l'effet de drogues, toxines, maladies musculaires (ex: myopathie) et neurodégénératives. Cette mesure de force est souvent employée en association avec le test de coordination motrice ROTAROD: un sujet présentant une coordination normale montrera des résultats médiocres en cas de faible force musculaire. Un must pour vos recherches sur l'activité, la coordination et le contrôle musculaire: particulièrement utile pour vos études sur les maladies de Parkinson et Huntington.
Nouveautés GS4 - 2023: Écran couleur rétroéclairé (meilleure lisibilité), pédale de remise à zéro, durée de batterie optimisée, taille de mémoire augmentée à 500 valeurs, compteur d'animaux, port USB (transfert de données/charge)

                    [thumb] => 
                    [img_empty] => /var/www/vhosts/de3310.ispfr.net/bioseb2024/modules/prestablog/views/img/product_link_white.jpg
                    [image_presente] => 1
                    [link] => https://bioseb.com/fr/activite-systeme-moteur-coordination/48-grip-strength-test.html
                )

        )

)
1