Journal
IN VIVO
Volume 36, Issue 2, Pages 610-617Publisher
INT INST ANTICANCER RESEARCH
DOI: 10.21873/invivo.12744
Keywords
Progranulin; Retinal pigment epithelium; lysosome
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Funding
- Nagai Memorial Research Scholarship from the Pharmaceutical Society of Japan
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The study found that deficiency of progranulin disrupts lysosomal activity and physiological functions in retinal pigment epithelium (RPE) cells. Additionally, loss of progranulin decreases cell viability and disrupts cell-cell junctions.
Background: Homozygous loss-of-function progranulin gene (GRN) mutation carriers develop adult onset neuronal ceroid lipofuscinosis (NCL), a lysosomal storage disease. Clinically, NCL patients display retinal degeneration and visual dysfunction. However, there is little information about the effects of progranulin dysfunction on lysosomal function of the retinal pigment epithelium (RPE). Materials and Methods: We performed RNA interference knock down of progranulin in primary human RPE (hRPE) cells and observed RPE function and lysosomal activity. Results: Progranulin localized to the lysosome in RPE cells. Loss of progranulin did not affect the biogenesis of lysosomes in RPE cells, while it was necessary for the activation of lysosomal proteases. Furthermore, progranulin deficiency decreased cell viability and disrupted the cell-cell junctions. Conclusion: Our results demonstrate that progranulin insufficiency disturbs lysosomal activity and physiological functions in RPE cells.
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