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Dernière publication 01/09/2009

Mitochondrial inhibitor 3-nitroproprionic acid enhances oxidative modification o

Multiple system atrophy (MSA) is a progressive neurodegenerative disease characterized by autonomic failure, parkinsonism, cerebellar ataxia, and...

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    [title] => Mitochondrial inhibitor 3-nitroproprionic acid enhances oxidative modification o
    [paragraph] => Mitochondrial inhibitor 3-nitroproprionic acid enhances oxidative modification of alpha-synuclein in a transgenic mouse model of multiple system atrophy.
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Authors
K. Ubhi, P. Hyu Lee, A. Adame, C. Inglis, M. Mante et al.

 Lab
University of California–San Diego, School of Medicine, Department of Neurosciences and Department of Pathology, La Jolla, California ; Yonsei University College of Medicine, Department of Neurology, Seoul, South Korea ; Innsbruck Medical University, Clin
Journal
Journal of Neuroscience Research
Abstract
Multiple system atrophy (MSA) is a progressive neurodegenerative disease characterized by autonomic failure, parkinsonism, cerebellar ataxia, and oligodendrocytic accumulation of alpha-synuclein (_syn). Oxidative stress has been linked to neuronal death in MSA and the mitochondrial toxin 3-nitropropionic acid (3NP) is known to enhance the motor deficits and neurodegeneration in transgenic mice models of MSA. However, the effect of 3NP administration on _syn itself has not been studied. In this context, we examined the neuropathological effects of 3NP administration in _syn transgenic mice expressing human _syn (h_syn) under the control of the myelin basic protein (MBP) promoter and the effect of this administration on posttranslational modifications of _syn, on levels of total _syn, and on its solubility. We demonstrate that 3NP administration altered levels of nitrated and oxidized _syn in the MBP-h_syn tg while not affecting global levels of phosphorylated or total _syn. 3NP administration also exaggerated neurological deficits in the MBP-h_syn tg mice, resulting in widespread neuronal degeneration and behavioral impairment.
BIOSEB Instruments Used
Grip strength test (BIO-GS3)
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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)

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