Journal
DEVELOPMENTAL DYNAMICS
Volume 252, Issue 1, Pages 61-80Publisher
WILEY
DOI: 10.1002/dvdy.516
Keywords
axon guidance dystrogly canneural migration synapse formation
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Glycoprotein dystroglycan plays a crucial role in connecting the extracellular matrix to the actin cytoskeleton in muscles, and also plays an important role in proper nervous system development. Animal models of dystroglycanopathy have been instrumental in uncovering the various roles of dystroglycan in neural circuit development.
The glycoprotein dystroglycan was first identified in muscle, where it functions as part of the dystrophin glycoprotein complex to connect the extracellular matrix to the actin cytoskeleton. Mutations in genes involved in the glycosylation of dystroglycan cause a form of congenital muscular dystrophy termed dystroglycanopathy. In addition to its well-defined role in regulating muscle integrity, dystroglycan is essential for proper central and peripheral nervous system development. Patients with dystroglycanopathy can present with a wide range of neurological perturbations, but unraveling the complex role of Dag1 in the nervous system has proven to be a challenge. Over the past two decades, animal models of dystroglycanopathy have been an invaluable resource that has allowed researchers to elucidate dystroglycan's many roles in neural circuit development. In this review, we summarize the pathways involved in dystroglycan's glycosylation and its known interacting proteins, and discuss how it regulates neuronal migration, axon guidance, synapse formation, and its role in non-neuronal cells.
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