Inherited retinal dystrophies: role of oxidative stress and inflammation in their physiopathology and therapeutic implications

Pinilla, Isabel (Universidad de Zaragoza) ; Maneu, Victoria ; Campello, Laura ; Fernández Sánchez, Laura ; Martínez Gil, Natalia ; Kutsyr, Oksana ; Sánchez Sáez, Xabier ; Sánchez Castillo, Carla ; Lax, Pedro ; Cuenca, Nicolás

Resumen: Inherited retinal dystrophies (IRDs) are a large group of genetically and clinically heteroge-neous diseases characterized by the progressive degeneration of the retina, ultimately leading to loss of visual function. Oxidative stress and inflammation play fundamental roles in the physiopathology of these diseases. Photoreceptor cell death induces an inflammatory state in the retina. The activation of several molecular pathways triggers different cellular responses to injury, including the activation of microglia to eliminate debris and recruit inflammatory cells from circulation. Therapeutical options for IRDs are currently limited, although a small number of patients have been successfully treated by gene therapy. Many other therapeutic strategies are being pursued to mitigate the deleterious effects of IRDs associated with oxidative metabolism and/or inflammation, including inhibiting reactive oxygen species’ accumulation and inflammatory responses, and blocking autophagy. Several compounds are being tested in clinical trials, generating great expectations for their implementation. The present review discusses the main death mechanisms that occur in IRDs and the latest therapies that are under investigation. © 2022 by the authors. Licensee MDPI, Basel, Switzerland.

Idioma: Inglés

DOI: 10.3390/antiox11061086

Año: 2022

Publicado en: Antioxidants 11, 6 (2022), 1086 [55 pp.]
ISSN: 2076-3921

Factor impacto JCR: 7.0 (2022)

Categ. JCR: BIOCHEMISTRY & MOLECULAR BIOLOGY rank: 46 / 285 = 0.161 (2022) - Q1 - T1
Categ. JCR: FOOD SCIENCE & TECHNOLOGY rank: 13 / 142 = 0.092 (2022) - Q1 - T1
Categ. JCR: CHEMISTRY, MEDICINAL rank: 6 / 60 = 0.1 (2022) - Q1 - T1

Factor impacto CITESCORE: 8.8 - Biochemistry, Genetics and Molecular Biology (Q1)

Factor impacto SCIMAGO: 1.084 - Biochemistry (Q1) - Clinical Biochemistry (Q1) - Food Science (Q1) - Physiology (Q1) - Molecular Biology (Q2) - Cell Biology (Q2)

Financiación: info:eu-repo/grantAgreement/ES/DGA/B08-17R
Financiación: info:eu-repo/grantAgreement/ES/ISCIII-FEDER/PI20-00740
Financiación: info:eu-repo/grantAgreement/ES/ISCIII/RETICS-FEDER-RD16-0008-0016
Financiación: info:eu-repo/grantAgreement/ES/MEC/FPU16-04114

Tipo y forma: Article (Published version)

Área (Departamento): Área Oftalmología (Dpto. Cirugía)