Requirement of enhanced Survival Motoneuron protein imposed during neuromuscular junction maturation
نویسندگان
چکیده
منابع مشابه
Requirement of enhanced Survival Motoneuron protein imposed during neuromuscular junction maturation.
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 ...
متن کاملMotoneuron survival is enhanced in the absence of neuromuscular junction formation in embryos.
Approximately half of the motoneurons produced during development die before birth or shortly after birth. Although it is believed that survival depends on a restricted supply of a trophic sustenance produced by the synaptic target tissue (i.e., muscle), it is unclear whether synapse formation per se is involved in motoneuron survival. To address this issue, we counted cranial motoneurons in a ...
متن کاملMaturation of the neuromuscular junction in masseters of human fetus.
OBJECTIVES The aim of the present investigation is to examine if the histological maturation of the neuromuscular junction in the masseters of human fetuses has already begun by the 12-th week of gestation or not. MATERIAL AND METHODS Twenty-four masseter muscles from 14 human fetuses at gestational age 12 weeks were divided into two groups. In the first group, muscle sections were stained wi...
متن کاملAggregation of sodium channels during development and maturation of the neuromuscular junction.
The voltage-activated Na channel (NaCh) is an integral membrane protein that is enriched at the neuromuscular end plate. Using loose-patch voltage-clamp and immunofluorescence, we have found that the aggregation of NaChs occurs late, during maturation of the neuromuscular junction. A decline in expression of embryonic NaCh mRNA and increase in adult NaCh mRNA precedes the onset of aggregation, ...
متن کاملPre- and postsynaptic maturation of the neuromuscular junction during neonatal synapse elimination depends on protein kinase C.
The distribution of acetylcholine receptors (AChRs) within and around the neuromuscular junction changes dramatically during the first postnatal weeks, a period during which polyneuronal innervation is eliminated. We reported previously that protein kinase C (PKC) activation accelerates postnatal synapse loss. Because of the close relationship between axonal retraction and AChR cluster dispersa...
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ژورنال
عنوان ژورنال: Journal of Clinical Investigation
سال: 2014
ISSN: 0021-9738
DOI: 10.1172/jci72017