Photoreceptor cells as a source of fundus autofluorescence in recessive Stargardt disease.
ABCA4
SD-OCT
bisretinoid
fundus autofluorescence
recessive Stargardt disease
retinal pigment epithelium
spectral domain optical coherence tomography
Journal
Journal of neuroscience research
ISSN: 1097-4547
Titre abrégé: J Neurosci Res
Pays: United States
ID NLM: 7600111
Informations de publication
Date de publication:
01 2019
01 2019
Historique:
received:
04
12
2017
revised:
12
03
2018
accepted:
10
04
2018
pubmed:
28
4
2018
medline:
12
6
2020
entrez:
28
4
2018
Statut:
ppublish
Résumé
Bisretinoid fluorophores form in photoreceptor outer segments from nonenzymatic reactions of vitamin A aldehyde. The short-wavelength autofluorescence (SW-AF) of fundus flecks in recessive Stargardt disease (STGD1) suggests a connection to these fluorophores. Through multimodal imaging, we sought to elucidate this link. Flecks observed in SW-AF images often colocalized with foci exhibiting reduced or absent near-infrared autofluorescence signal, the source of which is melanin in retinal pigment epithelial (RPE) cells. With serial imaging, changes in near-infrared autofluorescence (NIR-AF) preceded the onset of fleck hyperautofluorescence in SW-AF images and fleck profiles in NIR-AF images tended to be larger. Flecks in SW-AF and NIR-AF images also corresponded to hyperreflective lesions traversing photoreceptor-attributable bands in horizontal SD-OCT scans. The hyperreflective lesions interrupted adjacent OCT reflectivity bands and were associated with thinning of the outer nuclear layer. These SD-OCT findings are attributable to photoreceptor cell degeneration. Progressive increases and decreases in the SW-AF intensity of flecks were evident in color-coded quantitative fundus autofluorescence maps. In some cases, flecks appeared to spread radially from the fovea to approximately 8° of eccentricity, beyond which a circumferential spread characterized the distribution. Since the NIR-AF signal is derived from melanin and loss of this autofluorescence is indicative of RPE atrophy, the SW-AF of flecks cannot be accounted for by bisretinoid lipofuscin in RPE. Instead, we suggest that the bisretinoid serving as the source of the SW-AF signal, resides in photoreceptors, the cell that is also the site of bisretinoid synthesis.
Identifiants
pubmed: 29701254
doi: 10.1002/jnr.24252
pmc: PMC6532423
mid: NIHMS958863
doi:
Substances chimiques
Flecks
0
Lipofuscin
0
Zinc Phosphate Cement
7779-90-0
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Langues
eng
Sous-ensembles de citation
IM
Pagination
98-106Subventions
Organisme : NEI NIH HHS
ID : P30 EY019007
Pays : United States
Organisme : NEI NIH HHS
ID : R01 EY024091
Pays : United States
Organisme : NEI NIH HHS
ID : R01 EY028954
Pays : United States
Informations de copyright
© 2018 Wiley Periodicals, Inc.
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