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Activation of autophagy reverses progressive and deleterious protein aggregation in PRPF31 patient‐induced pluripotent stem cell‐derived retinal pigment epithelium cells
Journal article   Open access  Peer reviewed

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, …
Clinical and translational medicine, Vol.12(3), pp.e759-n/a
03/2022
DOI: https://doi.org/10.1002/ctm2.759
PMID: 35297555

Abstract

aggregate formation autophagy human pluripotent stem cells proteasome PRPF31 retinal organoids retinitis pigmentosa RPE tri‐snRNP assembly defects UPR
Schematic presentation shows the localisation of mutant PRPF31 in the cytoplasm 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.
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https://doi.org/10.1002/ctm2.759View
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