PARP inhibition preserves cone photoreceptors in rd2 retina

Abstract The rd2 mouse model, characterized by a mutation in the Prph2 gene, exhibits abnormal development of photoreceptor outer segments, resulting in progressive retinal degeneration. While the correlation between poly-ADP-ribose polymerase (PARP) activity and the degeneration of rod photorecepto...

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Main Authors: Pakize Nur Akkaya, María Miranda, Inmaculada Almansa, Cigdem Elmas, Dragana Trifunovic, Zohreh Hosseinzadeh, Ayse Sahaboglu
Format: Article
Language:English
Published: BMC 2025-04-01
Series:Acta Neuropathologica Communications
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Online Access:https://doi.org/10.1186/s40478-025-01982-5
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author Pakize Nur Akkaya
María Miranda
Inmaculada Almansa
Cigdem Elmas
Dragana Trifunovic
Zohreh Hosseinzadeh
Ayse Sahaboglu
author_facet Pakize Nur Akkaya
María Miranda
Inmaculada Almansa
Cigdem Elmas
Dragana Trifunovic
Zohreh Hosseinzadeh
Ayse Sahaboglu
author_sort Pakize Nur Akkaya
collection DOAJ
description Abstract The rd2 mouse model, characterized by a mutation in the Prph2 gene, exhibits abnormal development of photoreceptor outer segments, resulting in progressive retinal degeneration. While the correlation between poly-ADP-ribose polymerase (PARP) activity and the degeneration of rod photoreceptors is established in the rd2 model, the specific mechanism driving cone degeneration in this model remains unclear. Furthermore, it is yet to be determined whether inhibiting PARP activity can effectively impede the degeneration of cone photoreceptors in this context. We demonstrated that PARP inhibitors Olaparib, BMN-673, and 3-aminobenzamide (3AB), effectively reduced photoreceptor cell loss in the rd2 retina. Notably, rd2 retinas exhibited decreased cone density, but treatment with PARP inhibitors significantly protected cone photoreceptors. The PARP inhibitors, particularly BMN-673, demonstrated a significant protective effect as evidenced by increased rhodopsin expression within the outer segment and a concurrent decrease in Müller cell activity indicated by GFAP expression. The treatment also resulted in significant changes for markers of oxidative stress, such as glutathione (GSH), and oxidized glutathione (GSSG). Notably, the administration of PARP inhibitors also reduced CD9 expression (extracellular vesicle marker), which were significantly increased within the outer nuclear layer (ONL) in the rd2 retinas. Among PARP inhibitors, BMN-673 demonstrated the highest efficacy in preserving photoreceptors, particularly benefiting cone cells. Graphical Abstract
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spelling doaj-art-f4e9b61f1159453b84cd2a409cdc2a752025-08-20T03:04:54ZengBMCActa Neuropathologica Communications2051-59602025-04-0113111910.1186/s40478-025-01982-5PARP inhibition preserves cone photoreceptors in rd2 retinaPakize Nur Akkaya0María Miranda1Inmaculada Almansa2Cigdem Elmas3Dragana Trifunovic4Zohreh Hosseinzadeh5Ayse Sahaboglu6Department of Histology-Embryology, Balikesir University Faculty of MedicineDepartamento Ciencias Biomédicas, Facultad de Ciencias de La Salud, Universidad Cardenal Herrera-CEU, CEU UniversitiesDepartamento Ciencias Biomédicas, Facultad de Ciencias de La Salud, Universidad Cardenal Herrera-CEU, CEU UniversitiesDepartment of Histology-Embryology, Gazi University Faculty of MedicineCentre for Ophthalmology, Institute for Ophthalmic Research, Eberhard Karls UniversityDepartment of Ophthalmology, Radboud University Medical CenterCentre for Ophthalmology, Institute for Ophthalmic Research, Eberhard Karls UniversityAbstract The rd2 mouse model, characterized by a mutation in the Prph2 gene, exhibits abnormal development of photoreceptor outer segments, resulting in progressive retinal degeneration. While the correlation between poly-ADP-ribose polymerase (PARP) activity and the degeneration of rod photoreceptors is established in the rd2 model, the specific mechanism driving cone degeneration in this model remains unclear. Furthermore, it is yet to be determined whether inhibiting PARP activity can effectively impede the degeneration of cone photoreceptors in this context. We demonstrated that PARP inhibitors Olaparib, BMN-673, and 3-aminobenzamide (3AB), effectively reduced photoreceptor cell loss in the rd2 retina. Notably, rd2 retinas exhibited decreased cone density, but treatment with PARP inhibitors significantly protected cone photoreceptors. The PARP inhibitors, particularly BMN-673, demonstrated a significant protective effect as evidenced by increased rhodopsin expression within the outer segment and a concurrent decrease in Müller cell activity indicated by GFAP expression. The treatment also resulted in significant changes for markers of oxidative stress, such as glutathione (GSH), and oxidized glutathione (GSSG). Notably, the administration of PARP inhibitors also reduced CD9 expression (extracellular vesicle marker), which were significantly increased within the outer nuclear layer (ONL) in the rd2 retinas. Among PARP inhibitors, BMN-673 demonstrated the highest efficacy in preserving photoreceptors, particularly benefiting cone cells. Graphical Abstracthttps://doi.org/10.1186/s40478-025-01982-5rd2 retinasPhotoreceptor cell deathPARP inhibitionCone degenerationRetinal inflammation
spellingShingle Pakize Nur Akkaya
María Miranda
Inmaculada Almansa
Cigdem Elmas
Dragana Trifunovic
Zohreh Hosseinzadeh
Ayse Sahaboglu
PARP inhibition preserves cone photoreceptors in rd2 retina
Acta Neuropathologica Communications
rd2 retinas
Photoreceptor cell death
PARP inhibition
Cone degeneration
Retinal inflammation
title PARP inhibition preserves cone photoreceptors in rd2 retina
title_full PARP inhibition preserves cone photoreceptors in rd2 retina
title_fullStr PARP inhibition preserves cone photoreceptors in rd2 retina
title_full_unstemmed PARP inhibition preserves cone photoreceptors in rd2 retina
title_short PARP inhibition preserves cone photoreceptors in rd2 retina
title_sort parp inhibition preserves cone photoreceptors in rd2 retina
topic rd2 retinas
Photoreceptor cell death
PARP inhibition
Cone degeneration
Retinal inflammation
url https://doi.org/10.1186/s40478-025-01982-5
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AT cigdemelmas parpinhibitionpreservesconephotoreceptorsinrd2retina
AT draganatrifunovic parpinhibitionpreservesconephotoreceptorsinrd2retina
AT zohrehhosseinzadeh parpinhibitionpreservesconephotoreceptorsinrd2retina
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