prikaz prve stranice dokumenta The Surviving, Not Thriving, Photoreceptors in Patients with ABCA4 Stargardt Disease
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The Surviving, Not Thriving, Photoreceptors in Patients with ABCA4 Stargardt Disease
Diagnostics, 14 (2024), 14; 1545. https://doi.org/10.3390/diagnostics14141545
De Bruyn, Hanna; Johnson, Megan; Moretti, Madelyn; Ahmed, Saleh; Mujat, Mircea; Akula, James D.; Glavan, Tomislav; Mihalek, Ivana; Aslaksen, Sigrid; Molday, Laurie L.; Molday, Robert S.; Berkowitz, Bruce A.; Fulton, Anne B.

Citirajte ovaj rad

De Bruyn, H., Johnson, M., Moretti, M., Ahmed, S., Mujat, M., Akula, J. D. ... Fulton, A. B. (2024). The Surviving, Not Thriving, Photoreceptors in Patients with ABCA4 Stargardt Disease. Diagnostics, 14. (14). doi: 10.3390/diagnostics14141545

De Bruyn, Hanna, et al. "The Surviving, Not Thriving, Photoreceptors in Patients with ABCA4 Stargardt Disease." Diagnostics, vol. 14, br. 14, 2024. https://doi.org/10.3390/diagnostics14141545

De Bruyn, Hanna, Megan Johnson, Madelyn Moretti, Saleh Ahmed, Mircea Mujat, James D. Akula, Tomislav Glavan, et al. "The Surviving, Not Thriving, Photoreceptors in Patients with ABCA4 Stargardt Disease." Diagnostics 14, br. 14 (2024). https://doi.org/10.3390/diagnostics14141545

De Bruyn, H., et al. (2024) 'The Surviving, Not Thriving, Photoreceptors in Patients with ABCA4 Stargardt Disease', Diagnostics, 14(14). doi: 10.3390/diagnostics14141545

De Bruyn H, Johnson M, Moretti M, Ahmed S, Mujat M, Akula JD, i sur.. The Surviving, Not Thriving, Photoreceptors in Patients with ABCA4 Stargardt Disease. Diagnostics [Internet]. 17.07.2024. [pristupljeno 06.03.2025.];14(14). doi: 10.3390/diagnostics14141545

H. De Bruyn, et al., "The Surviving, Not Thriving, Photoreceptors in Patients with ABCA4 Stargardt Disease", Diagnostics, vol. 14, br. 14, Srpanj 2024. [Online]. Dostupno na: https://urn.nsk.hr/urn:nbn:hr:184:939255. [Citirano: 06.03.2025.]

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Naslov (engleski)The Surviving, Not Thriving, Photoreceptors in Patients with ABCA4 Stargardt Disease
AutorHanna De Bruyn
AutorMegan Johnson
AutorMadelyn Moretti
AutorSaleh Ahmed
AutorMircea Mujat
AutorJames D. Akula
AutorTomislav Glavan
AutorIvana Mihalek
AutorSigrid Aslaksen
AutorLaurie L. Molday
AutorRobert S. Molday
AutorBruce A. Berkowitz
AutorAnne B. Fulton
Autorova ustanovaSveučilište u Rijeci
Medicinski fakultet
(Katedra za molekularnu medicinu i biotehnologiju)
Znanstveno / umjetničko
područje, polje i grana		BIOMEDICINA I ZDRAVSTVO
Kliničke medicinske znanosti
Oftalmologija
Znanstveno / umjetničko
područje, polje i grana		BIOMEDICINA I ZDRAVSTVO
Temeljne medicinske znanosti
Genetika, genomika i proteomika čovjeka
Sažetak (engleski)
Stargardt disease (STGD1), associated with biallelic variants in the ABCA4 gene, is the most common heritable macular dystrophy and is currently untreatable. To identify potential treatment targets, we characterized surviving STGD1 photoreceptors. We used clinical data to identify macular regions with surviving STGD1 photoreceptors. We compared the hyperreflective bands in the optical coherence tomographic (OCT) images that correspond to structures in the STGD1 photoreceptor inner segments to those in controls. We used adaptive optics scanning light ophthalmoscopy (AO-SLO) to study the distribution of cones and AO-OCT to evaluate the interface of photoreceptors and retinal pigment epithelium (RPE). We found that the profile of the hyperreflective bands differed dramatically between patients with STGD1 and controls. AO-SLOs showed patches in which cone densities were similar to those in healthy retinas and others in which the cone population was sparse. In regions replete with cones, there was no debris at the photoreceptor-RPE interface. In regions with sparse cones, there was abundant debris. Our results raise the possibility that pharmaceutical means may protect surviving photoreceptors and so mitigate vision loss in patients with STGD1.
Ključne riječi (engleski)
Jezikengleski
Vrsta publikacijeZnanstveni rad - Izvorni znanstveni rad
Status objaveObjavljen
Vrsta recenzijeRecenziran - međunarodna recenzija
Verzija publikacijeObjavljena verzija rada (izdavačev PDF)
Naslov časopisaDiagnostics
Brojčani podacivol. 14, br. 14, 1545
e-ISSN2075-4418
DOIhttps://doi.org/10.3390/diagnostics14141545
URN:NBNurn:nbn:hr:184:939255
Datum objave publikacije2024-07-17
URL dokumentahttps://www.mdpi.com/2075-4418/14/14/1545
Vrsta resursaTekst
Prava pristupaOtvoreni pristup
Uvjeti korištenjaIkona licence Imenovanje 4.0 međunarodna (CC BY 4.0)
Datum i vrijeme pohrane2024-08-08 07:23:00