Activation of autophagy reverses progressive and deleterious protein aggregation in PRPF31 patient‐induced pluripotent stem cell‐derived retinal pigment epithelium cells

Maria Georgiou; Chunbo Yang; Robert Atkinson; Kuan‐Ting Pan; Adriana Buskin; Marina Moya Molina; Joseph Collin; Jumana Al‐Aama; Franziska Goertler; Sebastian E. J. Ludwig; Tracey Davey; Reinhard Lührmann; Sushma Nagaraja‐Grellscheid; Colin A. Johnson; Robin Ali; Lyle Armstrong; Viktor Korolchuk; Henning Urlaub; Sina Mozaffari‐Jovin; Majlinda Lako

Journal ArticleOPEN ACCESS

Activation of autophagy reverses progressive and deleterious protein aggregation in PRPF31 patient‐induced pluripotent stem cell‐derived retinal pigment epithelium cells

Georgiou M
Yang C
Atkinson R
et al.

Clinical and Translational Medicine (2022) 12(3)

DOI: 10.1002/ctm2.759

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Abstract

Graphical Abstract Schematic presentation shows the localisation of mutant PRPF31 in the cyto-plasm of RP11-RPE cells and accumulation of aggregates containing HSPs, visual cycle and ubiquitin conjugated proteins, which were much reduced upon application of autophagy activator, Rapamycin. m-mutant PRPF31, CB-Cajal bodies, Ub-ubiquitin, HSPs-heat shock proteins. Clin. Transl. Med. 2022;12:e759. wileyonlinelibrary.com/journal/ctm2 https://doi.

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Georgiou, M., Yang, C., Atkinson, R., Pan, K., Buskin, A., Molina, M. M., … Lako, M. (2022). Activation of autophagy reverses progressive and deleterious protein aggregation in PRPF31 patient‐induced pluripotent stem cell‐derived retinal pigment epithelium cells. Clinical and Translational Medicine, 12(3). https://doi.org/10.1002/ctm2.759

Activation of autophagy reverses progressive and deleterious protein aggregation in PRPF31 patient‐induced pluripotent stem cell‐derived retinal pigment epithelium cells

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