Publications

Dernière publication 28/02/2017

Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis o

Motor neuron-extrinsic mechanisms have been shown to participate in the pathogenesis of ALS-SOD1, one familial form of amyotrophic lateral...

Array
(
    [id_prestablog_news] => 1145
    [id_shop] => 1
    [date] => 2017-02-28 00:00:00
    [date_modification] => 2024-02-09 14:15:12
    [langues] => ["1","2"]
    [actif] => 1
    [slide] => 0
    [url_redirect] => 
    [average_rating] => 
    [number_rating] => 
    [author_id] => 1
    [featured] => 0
    [prim_key] => 2250
    [id_lang] => 2
    [title] => Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis o
    [paragraph] => Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS_associated amyotrophic lateral sclerosis 
    [content] => 
Authors
J Scekic_Zahirovic, H El Oussini, S Mersmann et al

 Lab
Inserm, UMR-S1118, Strasbourg, France
Journal
Acta Neuropathologica
Abstract
Motor neuron-extrinsic mechanisms have been shown to participate in the pathogenesis of ALS-SOD1, one familial form of amyotrophic lateral sclerosis (ALS). It remains unclear whether such mechanisms contribute to other familial forms, such as TDP-43 and FUS-associated ALS. Here, we characterize a single-copy mouse model of ALS-FUS that conditionally expresses a disease-relevant truncating FUS mutant from the endogenous murine Fus gene. We show that these mice, but not mice heterozygous for a Fus null allele, develop similar pathology as ALS-FUS patients and a mild motor neuron phenotype. Most importantly, CRE-mediated rescue of the Fus mutation within motor neurons prevented degeneration of motor neuron cell bodies, but only delayed appearance of motor symptoms. Indeed, we observed downregulation of multiple myelin-related genes, and increased numbers of oligodendrocytes in the spinal cord supporting their contribution to behavioral deficits. In all, we show that mutant FUS triggers toxic events in both motor neurons and neighboring cells to elicit motor neuron disease.
BIOSEB Instruments Used
Grip strength test (BIO-GS3)
    [meta_description] => 
    [meta_keywords] => https://link.springer.com/article/10.1007%2Fs00401-017-1687-9
    [meta_title] => 
    [link_rewrite] => motor-neuron-intrinsic-and-extrinsic-mechanisms-contribute-to-the-pathogenesis-of-fusassociated-amyotrophic-lateral-sclerosis-
    [actif_langue] => 1
    [read] => 848
    [count_comments] => 0
    [id] => 1145
    [categories] => Array
        (
            [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
                )

        )

    [authors] => 
    [paragraph_crop] => Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of [...]
    [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/preprod.bioseb.com/modules/prestablog/views/img/product_link_white.jpg
                    [image_presente] => 1
                    [link] => https://preprod.bioseb.com/fr/activite-systeme-moteur-coordination/48-grip-strength-test.html
                )

        )

)
1