Widespread subclinical cellular changes revealed across a neural-epithelial-vascular complex in choroideremia using adaptive optics.
Journal
Communications biology
ISSN: 2399-3642
Titre abrégé: Commun Biol
Pays: England
ID NLM: 101719179
Informations de publication
Date de publication:
13 09 2022
13 09 2022
Historique:
received:
21
04
2022
accepted:
12
08
2022
entrez:
13
9
2022
pubmed:
14
9
2022
medline:
16
9
2022
Statut:
epublish
Résumé
Choroideremia is an X-linked, blinding retinal degeneration with progressive loss of photoreceptors, retinal pigment epithelial (RPE) cells, and choriocapillaris. To study the extent to which these layers are disrupted in affected males and female carriers, we performed multimodal adaptive optics imaging to better visualize the in vivo pathogenesis of choroideremia in the living human eye. We demonstrate the presence of subclinical, widespread enlarged RPE cells present in all subjects imaged. In the fovea, the last area to be affected in choroideremia, we found greater disruption to the RPE than to either the photoreceptor or choriocapillaris layers. The unexpected finding of patches of photoreceptors that were fluorescently-labeled, but structurally and functionally normal, suggests that the RPE blood barrier function may be altered in choroideremia. Finally, we introduce a strategy for detecting enlarged cells using conventional ophthalmic imaging instrumentation. These findings establish that there is subclinical polymegathism of RPE cells in choroideremia.
Identifiants
pubmed: 36100689
doi: 10.1038/s42003-022-03842-7
pii: 10.1038/s42003-022-03842-7
pmc: PMC9470576
doi:
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Research Support, N.I.H., Intramural
Langues
eng
Sous-ensembles de citation
IM
Pagination
893Subventions
Organisme : NEI NIH HHS
ID : P30 EY026877
Pays : United States
Organisme : NEI NIH HHS
ID : R01 EY031360
Pays : United States
Organisme : NEI NIH HHS
ID : R01 EY032147
Pays : United States
Organisme : NEI NIH HHS
ID : R01 EY032669
Pays : United States
Informations de copyright
© 2022. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.
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