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Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF

Caroni, Pico ; Pun, San ; Santos, Alexandre Ferrão ; Saxena, Smita ; Xu, Lan

Nature neuroscience, 2006-03, Vol.9 (3), p.408-419 [Periódico revisado por pares]

United States: Nature Publishing Group

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Título:
Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF
Autor: Caroni, Pico ; Pun, San ; Santos, Alexandre Ferrão ; Saxena, Smita ; Xu, Lan
Assuntos: Amyotrophic lateral sclerosis ; Animals ; Axons ; Axons - drug effects ; Axons - metabolism ; Axons - pathology ; Brain research ; Ciliary neurotrophic factor ; Ciliary Neurotrophic Factor - pharmacology ; Ciliary Neurotrophic Factor - therapeutic use ; Degeneration ; Denervation ; Disease ; Disease Models, Animal ; Genetic Predisposition to Disease - genetics ; Humans ; Identification and classification ; Mice ; Mice, Transgenic ; Motor Neuron Disease - drug therapy ; Motor Neuron Disease - genetics ; Motor Neuron Disease - physiopathology ; Motor Neurons - drug effects ; Motor Neurons - metabolism ; Motor Neurons - pathology ; Muscle Fatigue - drug effects ; Muscle Fatigue - genetics ; Muscle Fibers, Fast-Twitch - physiology ; Muscle Fibers, Slow-Twitch - physiology ; Muscle strength ; Muscle, Skeletal - innervation ; Muscle, Skeletal - physiopathology ; Mutation - genetics ; Nerve Degeneration - drug therapy ; Nerve Degeneration - genetics ; Nerve Degeneration - physiopathology ; Nervous system ; Neuromuscular Junction - metabolism ; Neuromuscular Junction - ultrastructure ; Neuronal Plasticity - genetics ; Physiological aspects ; Superoxide Dismutase - genetics ; Superoxide Dismutase-1 ; Synaptic Vesicles - metabolism ; Synaptic Vesicles - ultrastructure ; Topography ; Transgenic animals
É parte de: Nature neuroscience, 2006-03, Vol.9 (3), p.408-419
Notas: ObjectType-Article-1
SourceType-Scholarly Journals-1
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Descrição: Neurodegenerative diseases can have long preclinical phases and insidious progression patterns, but the mechanisms of disease progression are poorly understood. Because quantitative accounts of neuronal circuitry affected by disease have been lacking, it has remained unclear whether disease progression reflects processes of stochastic loss or temporally defined selective vulnerabilities of distinct synapses or axons. Here we derive a quantitative topographic map of muscle innervation in the hindlimb. We show that in two mouse models of motoneuron disease (G93A SOD1 and G85R SOD1), axons of fast-fatiguable motoneurons are affected synchronously, long before symptoms appear. Fast-fatigue-resistant motoneuron axons are affected at symptom-onset, whereas axons of slow motoneurons are resistant. Axonal vulnerability leads to synaptic vesicle stalling and accumulation of BC12a1-a, an anti-apoptotic protein. It is alleviated by ciliary neurotrophic factor and triggers proteasome-dependent pruning of peripheral axon branches. Thus, motoneuron disease involves predictable, selective vulnerability patterns by physiological subtypes of axons, episodes of abrupt pruning in the target region and compensation by resistant axons.
Editor: United States: Nature Publishing Group
Idioma: Inglês

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Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF

Caroni, Pico ; Pun, San ; Santos, Alexandre Ferrão ; Saxena, Smita ; Xu, Lan

United States: Nature Publishing Group

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