Motor neuron pathology and behavioral alterations at late stages in a SMA mouse model

Abstract Spinal muscular atrophy (SMA) is a neurogenetic autosomal recessive disorder characterized by degeneration of lower motor neurons. The validation of appropriate animal models is key in fostering SMA research. Recent studies set up an animal model showing long survival and slow disease progr...

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Veröffentlicht in:	Brain research 2012-03, Vol.1442, p.66-75
Hauptverfasser:	Fulceri, Federica, Bartalucci, Alessia, Paparelli, Silvio, Pasquali, Livia, Biagioni, Francesca, Ferrucci, Michela, Ruffoli, Riccardo, Fornai, Francesco
Format:	Artikel
Sprache:	eng
Schlagworte:	animal models Animals brain Cell Size disease course Disease Models, Animal Disease Progression drugs genes genetically modified organisms Humans Mice Mice, Knockout Mice, Transgenic Motor Activity Motor neuron disease Motor neuron heterotopy Motor neuron size motor neurons Motor Neurons - pathology Muscle Weakness muscular atrophy Muscular Atrophy, Spinal - pathology Muscular Atrophy, Spinal - physiopathology mutation Nerve Degeneration Neurology patients Reflex Rotarod Performance Test spinal cord Spinal Cord - pathology Spinal muscular atrophy Survival Motor Neuron protein Survival of Motor Neuron 1 Protein - genetics Survival of Motor Neuron 2 Protein - genetics therapeutics
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