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Widespread subclinical cellular changes revealed across a neural-epithelial-vascular complex in choroideremia using adaptive optics.
Aguilera, Nancy; Liu, Tao; Bower, Andrew J; Li, Joanne; Abouassali, Sarah; Lu, Rongwen; Giannini, John; Pfau, Maximilian; Bender, Chelsea; Smelkinson, Margery G; Naik, Amelia; Guan, Bin; Schwartz, Owen; Volkov, Andrei; Dubra, Alfredo; Liu, Zhuolin; Hammer, Daniel X; Maric, Dragan; Fariss, Robert; Hufnagel, Robert B; Jeffrey, Brett G; Brooks, Brian P; Zein, Wadih M; Huryn, Laryssa A; Tam, Johnny.
Afiliação
  • Aguilera N; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Liu T; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Bower AJ; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Li J; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Abouassali S; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Lu R; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Giannini J; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Pfau M; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Bender C; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Smelkinson MG; National Institute of Allergy and Infectious Disease, Research Technologies Branch, National Institutes of Health, Bethesda, MD, USA.
  • Naik A; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Guan B; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Schwartz O; National Institute of Allergy and Infectious Disease, Research Technologies Branch, National Institutes of Health, Bethesda, MD, USA.
  • Volkov A; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Dubra A; Department of Ophthalmology, Stanford University, Palo Alto, CA, USA.
  • Liu Z; Division of Biomedical Physics, Office of Science and Engineering Laboratories, Center for Devices and Radiological Health, Food and Drug Administration, Silver Spring, MD, USA.
  • Hammer DX; Division of Biomedical Physics, Office of Science and Engineering Laboratories, Center for Devices and Radiological Health, Food and Drug Administration, Silver Spring, MD, USA.
  • Maric D; National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA.
  • Fariss R; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Hufnagel RB; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Jeffrey BG; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Brooks BP; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Zein WM; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Huryn LA; National Eye Institute, National Institutes of Health, Bethesda, MD, USA.
  • Tam J; National Eye Institute, National Institutes of Health, Bethesda, MD, USA. johnny@nih.gov.
Commun Biol ; 5(1): 893, 2022 09 13.
Article em En | MEDLINE | ID: mdl-36100689
ABSTRACT
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.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Degeneração Retiniana / Coroideremia Limite: Female / Humans / Male Idioma: En Ano de publicação: 2022 Tipo de documento: Article
Texto completo
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Degeneração Retiniana / Coroideremia Limite: Female / Humans / Male Idioma: En Ano de publicação: 2022 Tipo de documento: Article