Publications

Dernière publication 27/01/2014

Requirement of enhanced Survival Motoneuron protein imposed during neuromuscular

Spinal muscular atrophy is a common motor neuron disease caused by low survival motoneuron (SMN), a key protein in the proper splicing of genes....

Array
(
    [id_prestablog_news] => 605
    [id_shop] => 1
    [date] => 2014-01-27 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
    [prim_key] => 1186
    [id_lang] => 2
    [title] => Requirement of enhanced Survival Motoneuron protein imposed during neuromuscular
    [paragraph] => Requirement of enhanced Survival Motoneuron protein imposed during neuromuscular junction maturation
    [content] => 
Authors
S. Kariya, T. Obis, C. Garone, T. Akay, F. Sera et al.

 Lab
Columbia University Medical Center, New York, USA
Journal
Journal of Clinical Investigation
Abstract
Spinal muscular atrophy is a common motor neuron disease caused by low survival motoneuron (SMN), a key protein in the proper splicing of genes. Restoring the protein is therefore a promising therapeutic strategy. Implementation of this strategy, however, depends on defining the temporal requirements for SMN. Here, we used controlled knockdown of SMN in transgenic mice to determine the precise postnatal stage requirements for this protein. Reducing SMN in neonatal mice resulted in a classic SMA-like phenotype. Unexpectedly, depletion of SMN in adults had relatively little effect. Insensitivity to low SMN emerged abruptly at postnatal day 17, which coincided with establishment of the fully mature neuromuscular junction (NMJ). Mature animals depleted of SMN eventually exhibited evidence of selective neuromuscular pathology that was made worse by traumatic injury. The ability to regenerate the mature NMJ in aged or injured SMN-depleted mice was grossly impaired, a likely consequence of the inability to meet the surge in demand for motoneuronal SMN that was seen in controls. Our results demonstrate that relative maturity of the NMJ determines the temporal requirement for the SMN protein. These observations suggest that the use of potent but potentially deleterious SMN-enhancing agents could be tapered in human patients once the neuromuscular system matures and reintroduced as needed to enhance SMN for remodeling aged or injured NMJs.
BIOSEB Instruments Used
Grip strength test (BIO-GS3)
    [meta_description] => 
    [meta_keywords] => http://www.jci.org/articles/view/72017#sd
    [meta_title] => 
    [link_rewrite] => requirement-of-enhanced-survival-motoneuron-protein-imposed-during-neuromuscular-junction-maturation
    [actif_langue] => 1
    [read] => 941
    [count_comments] => 0
    [id] => 605
    [categories] => Array
        (
            [53] => Array
                (
                    [id_prestablog_categorie] => 53
                    [title] => Functions de motricité générale
                    [link_rewrite] => Functions-de-motricite-generale
                )

            [92] => Array
                (
                    [id_prestablog_categorie] => 92
                    [title] => Petite enfance
                    [link_rewrite] => Petite-enfance
                )

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

            [14] => Array
                (
                    [id_prestablog_categorie] => 14
                    [title] => Système moteur
                    [link_rewrite] => Systeme-moteur
                )

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

        )

    [authors] => 
    [paragraph_crop] => Requirement of enhanced Survival Motoneuron protein imposed during neuromuscular junction maturation
    [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