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Validating the RedMIT/GFP-LC3 Mouse Model by Studying Mitophagy in Autosomal Dominant Optic Atrophy Due to the OPA1Q285STOP Mutation

Background: Autosomal dominant optic atrophy (ADOA) is usually caused by mutations in the essential gene, OPA1. This encodes a ubiquitous protein involved in mitochondrial dynamics, hence tissue specificity is not understood. Dysregulated mitophagy (mitochondria recycling) is implicated in ADOA, bei...

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Detalles Bibliográficos
Autores principales: Diot, Alan, Agnew, Thomas, Sanderson, Jeremy, Liao, Chunyan, Carver, Janet, Neves, Ricardo Pires das, Gupta, Rajeev, Guo, Yanping, Waters, Caroline, Seto, Sharon, Daniels, Matthew J., Dombi, Eszter, Lodge, Tiffany, Morten, Karl, Williams, Suzannah A., Enver, Tariq, Iborra, Francisco J., Votruba, Marcela, Poulton, Joanna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6156146/
https://www.ncbi.nlm.nih.gov/pubmed/30283778
http://dx.doi.org/10.3389/fcell.2018.00103