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Gonneke S. K. Pilgram Saranyapin Potikanond Richard A. Baines Lee G. Fradkin Jasprina N. Noordermeer 《Molecular neurobiology》2010,41(1):1-21
Duchenne muscular dystrophy is caused by mutations in the dystrophin gene and is characterized by progressive muscle wasting. A number of Duchenne patients also present with mental retardation.
The dystrophin protein is part of the highly conserved dystrophin-associated glycoprotein complex (DGC) which accumulates
at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems. Many years
of research into the roles of the DGC in muscle have revealed its structural function in stabilizing the sarcolemma. In addition,
the DGC also acts as a scaffold for various signaling pathways. Here, we discuss recent advances in understanding DGC roles
in the nervous system, gained from studies in both vertebrate and invertebrate model systems. From these studies, it has become
clear that the DGC is important for the maturation of neurotransmitter receptor complexes and for the regulation of neurotransmitter
release at the NMJ and central synapses. Furthermore, roles for the DGC have been established in consolidation of long-term
spatial and recognition memory. The challenges ahead include the integration of the behavioral and mechanistic studies and
the use of this information to identify therapeutic targets. 相似文献
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Salinee Jantrapirom Wutigri Nimlamool Piya Temviriyanukul Somaieh Ahmadian Cody J. Locke Graeme W. Davis Masamitsu Yamaguchi Jasprina N. Noordermeer Lee G. Fradkin Saranyapin Potikanond 《生物化学与生物物理学报:疾病的分子基础》2019,1865(6):1579-1591
Evolutionarily conserved homeostatic systems have been shown to modulate synaptic efficiency at the neuromuscular junctions of organisms. While advances have been made in identifying molecules that function presynaptically during homeostasis, limited information is currently available on how postsynaptic alterations affect presynaptic function. We previously identified a role for postsynaptic Dystrophin in the maintenance of evoked neurotransmitter release. We herein demonstrated that Dystrobrevin, a member of the Dystrophin Glycoprotein Complex, was delocalized from the postsynaptic region in the absence of Dystrophin. A newly-generated Dystrobrevin mutant showed elevated evoked neurotransmitter release, increased bouton numbers, and a readily releasable pool of synaptic vesicles without changes in the function or numbers of postsynaptic glutamate receptors. In addition, we provide evidence to show that the highly conserved Cdc42 Rho GTPase plays a key role in the postsynaptic Dystrophin/Dystrobrevin pathway for synaptic homeostasis. The present results give novel insights into the synaptic deficits underlying Duchenne Muscular Dystrophy affected by a dysfunctional Dystrophin Glycoprotein complex. 相似文献
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