在表达Musk/Trk嵌合受体的Musk突变小鼠中突触形成的恢复。

Restoration of synapse formation in Musk mutant mice expressing a Musk/Trk chimeric receptor.

作者信息

Herbst Ruth, Avetisova Ekaterina, Burden Steven J

机构信息

Molecular Neurobiology Program, Skirball Institute, NYU Medical School, NY 10016, USA.

出版信息

Development. 2002 Dec;129(23):5449-60. doi: 10.1242/dev.00112.

Abstract

Mice lacking Musk, a muscle-specific receptor tyrosine kinase that is activated by agrin, fail to form neuromuscular synapses and consequently die at birth because of their failure to move or breathe. We produced mice that express a chimeric receptor, containing the juxtamembrane region of Musk and the kinase domain of TrkA, selectively in muscle, and we crossed this transgene into Musk mutant mice. Expression of this chimeric receptor restores presynaptic and postsynaptic differentiation, including the formation of nerve terminal arbors, synapse-specific transcription, and clustering of postsynaptic proteins, allowing Musk mutant mice to move, breathe and survive as adults. These results show that the juxtamembrane region of Musk, including a single phosphotyrosine docking site, even in the context of a different kinase domain, is sufficient to activate the multiple pathways leading to presynaptic and postsynaptic differentiation in vivo. In addition, we find that Musk protein can be clustered at synaptic sites, even if Musk mRNA is expressed uniformly in muscle. Moreover, acetylcholine receptor clustering and motor terminal branching are restored in parallel, indicating that the extent of presynaptic differentiation is matched to the extent of postsynaptic differentiation.

摘要

缺乏Musk(一种由聚集蛋白激活的肌肉特异性受体酪氨酸激酶)的小鼠无法形成神经肌肉突触,因此由于无法移动或呼吸而在出生时死亡。我们培育了在肌肉中选择性表达嵌合受体的小鼠,该嵌合受体包含Musk的近膜区域和TrkA的激酶结构域,并将此转基因导入Musk突变小鼠中。这种嵌合受体的表达恢复了突触前和突触后的分化,包括神经末梢分支的形成、突触特异性转录以及突触后蛋白的聚集,使Musk突变小鼠能够像成年小鼠一样移动、呼吸并存活。这些结果表明,即使在不同激酶结构域的情况下,Musk的近膜区域(包括单个磷酸酪氨酸对接位点)也足以激活体内导致突触前和突触后分化的多种途径。此外,我们发现即使Musk mRNA在肌肉中均匀表达,Musk蛋白也能聚集在突触部位。而且,乙酰胆碱受体聚集和运动终末分支同时恢复,表明突触前分化的程度与突触后分化的程度相匹配。

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