SMN requirement for synaptic vesicle, active zone and microtubule postnatal organization in motor nerve terminals

Low levels of the Survival Motor Neuron (SMN) protein produce Spinal Muscular Atrophy (SMA), a severe monogenetic disease in infants characterized by muscle weakness and impaired synaptic transmission. We report here severe structural and functional alterations in the organization of the organelles...

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Veröffentlicht in:	PloS one 2011-10, Vol.6 (10), p.e26164-e26164
Hauptverfasser:	Torres-Benito, Laura, Neher, Margret Feodora, Cano, Raquel, Ruiz, Rocio, Tabares, Lucia
Format:	Artikel
Sprache:	eng
Schlagworte:	Actins - metabolism Analysis Animals Animals, Newborn Atrophy Bassoon music Biology Biophysics Cell survival Cluster Analysis Clustering Cytoskeleton Functional morphology Growth factors Hypotheses Infants Maturation Medicine Mice Microtubules - metabolism Microtubules - pathology Mitochondria Mitochondria - metabolism Motility Motor Neurons - metabolism Motor Neurons - pathology Muscular Atrophy, Spinal - metabolism Nerve endings Nerve Endings - metabolism Nerve Endings - pathology Neurodegeneration Neuromuscular diseases Neurons Organelles Pediatric diseases Physiology Proteins Rodents SMN protein Spinal muscular atrophy Structure-function relationships Survival of Motor Neuron 1 Protein - metabolism Synaptic transmission Synaptic vesicles Synaptic Vesicles - metabolism Synaptic Vesicles - pathology Terminals Vesicles
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