Strochlic Laure, Cartaud Annie, Cartaud Jean
Biologie Cellulaire des Membranes, Institut Jacques Monod, Paris, France.
Bioessays. 2005 Nov;27(11):1129-35. doi: 10.1002/bies.20305.
The muscle-specific kinase MuSK is part of an agrin receptor complex that stimulates tyrosine phosphorylation and drives clustering of acetylcholine receptors (AChRs) in the postsynaptic membrane at the vertebrate neuromuscular junction (NMJ). MuSK also regulates synaptic gene transcription in subsynaptic nuclei. Over the past few years, decisive progress has been made in the identification of MuSK effectors, helping to understand its function in the formation of the NMJ. Similarly to AChR, MuSK and several of its partners are the target of mutations responsible for diseases of the NMJ, such as congenital myasthenic syndromes. This minireview will focus on the multiple MuSK effectors so far identified that place MuSK at the center of a multifunctional signaling complex involved in the organization of the NMJ and associated disorders.
肌肉特异性激酶MuSK是聚集蛋白受体复合物的一部分，该复合物可刺激酪氨酸磷酸化，并驱动脊椎动物神经肌肉接头（NMJ）突触后膜中乙酰胆碱受体（AChR）的聚集。MuSK还调节突触下细胞核中的突触基因转录。在过去几年中，在鉴定MuSK效应器方面取得了决定性进展，这有助于理解其在NMJ形成中的作用。与AChR类似，MuSK及其几个伴侣是导致NMJ疾病（如先天性肌无力综合征）的突变靶点。本综述将聚焦于目前已鉴定出的多种MuSK效应器，这些效应器使MuSK处于参与NMJ组织及相关疾病的多功能信号复合物的中心位置。
