The many roles of dystroglycan in nervous system development and function: Dystroglycan and neural circuit development: Dystroglycan and neural circuit development.


Journal

Developmental dynamics : an official publication of the American Association of Anatomists
ISSN: 1097-0177
Titre abrégé: Dev Dyn
Pays: United States
ID NLM: 9201927

Informations de publication

Date de publication:
01 2023
Historique:
revised: 21 06 2022
received: 09 05 2022
accepted: 22 06 2022
pubmed: 1 7 2022
medline: 4 1 2023
entrez: 30 6 2022
Statut: ppublish

Résumé

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.

Identifiants

pubmed: 35770940
doi: 10.1002/dvdy.516
doi:

Substances chimiques

Dystroglycans 146888-27-9
Glycoproteins 0

Types de publication

Journal Article Review Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

61-80

Subventions

Organisme : NINDS NIH HHS
ID : F31 NS120649
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS091027
Pays : United States

Informations de copyright

© 2022 American Association for Anatomy.

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Auteurs

Jennifer N Jahncke (JN)

Neuroscience Graduate Program, Oregan Health & Science University, Portland, Oregon, USA.

Kevin M Wright (KM)

Vollum Institute, Oregon Health & Science University, Portland, Oregon, USA.

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