Dystrophin Is Required for the Proper Timing in Retinal Histogenesis: A Thorough Investigation on the
Duchenne muscular dystrophy
GABA
amacrine cells
dystrophin
mdx mice
retinal ganglion cells
retinogenesis
Journal
Frontiers in neuroscience
ISSN: 1662-4548
Titre abrégé: Front Neurosci
Pays: Switzerland
ID NLM: 101478481
Informations de publication
Date de publication:
2020
2020
Historique:
received:
25
04
2020
accepted:
29
06
2020
entrez:
28
9
2020
pubmed:
29
9
2020
medline:
29
9
2020
Statut:
epublish
Résumé
Duchenne muscular dystrophy (DMD) is a lethal X-linked muscular disease caused by defective expression of the cytoskeletal protein dystrophin (Dp427). Selected autonomic and central neurons, including retinal neurons, express Dp427 and/or dystrophin shorter isoforms. Because of this, DMD patients may also experience different forms of cognitive impairment, neurological and autonomic disorders, and specific visual defects. DMD-related damages to the nervous system are established during development, suggesting a role for all dystrophin isoforms in neural circuit development and differentiation; however, to date, their function in retinogenesis has never been investigated. In this large-scale study, we analyzed whether the lack of Dp427 affects late retinogenesis in the
Identifiants
pubmed: 32982660
doi: 10.3389/fnins.2020.00760
pmc: PMC7487415
doi:
Types de publication
Journal Article
Langues
eng
Pagination
760Informations de copyright
Copyright © 2020 Persiconi, Cosmi, Guadagno, Lupo and De Stefano.
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