Journal
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
Volume 22, Issue 21, Pages -Publisher
MDPI
DOI: 10.3390/ijms222111557
Keywords
progranulin; retina; retinal pigment epithelium; microglia; lysosome; neuronal ceroid lipofuscinosis
Funding
- Nagai Memorial Research Scholarship from the Pharmaceutical Society of Japan
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This study characterized the retinal phenotype in mature PGRN knockout mice, showing reduced a-wave amplitude and outer nuclear thickness in Grn(-/-) mice compared to wild-type mice. Increased accumulation of retinal microglial cells on the RPE apical layer and changes in lysosomal protein expression were observed in Grn(-/-) mice, indicating a potential modulation of microglial dynamics in PGRN deficiency.
Progranulin (PGRN) is a secreted glycoprotein that regulates numerous cellular processes. The role of PGRN as a regulator of lysosomes has recently received attention. The purpose of this study was to characterize the retinal phenotype in mature PGRN knockout (Grn(-/-)) mice. The a-wave amplitude of scotopic electroretinogram and outer nuclear thickness were significantly reduced at 6 months of age in Grn(-/-) mice compared to wild-type (Grn(+/+)) mice. In Grn(-/-) mice, retinal microglial cells accumulated on the retinal pigment epithelium (RPE) apical layer, and the number of infiltrated microglia and white fundus lesions between 2 and 6 months of age showed a close affinity. In Grn(+/+) mice, PGRN was located in the retina, while the strongest PGRN signals were detected in the RPE-choroid. The different effects of PGRN deficiency on the expression of lysosomal proteins between the retina and RPE-choroid were demonstrated. Our data suggest that the subretinal translocation of microglia is a characteristic phenotype in the retina of mature PGRN knockout mice. The different effects of PGRN deficiency on the expression of lysosomal proteins between the retina and RPE-choroid might modulate microglial dynamics in PGRN knockout mice.
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