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Altered Regulation of Ion Channels in Cultured Retinal Pigment Epithelial Cells From Rcs Rats

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Abstract

The Royal College of Surgeon (RCS) rat suffers from an inherited retinal degeneration because their retinal pigment epithelium (RPE) is unable to phagocytose shed photoreceptor outer membranes (1,2,3,4). The genetical cause leading to this functional defect is unknown. The phagocytosis of photoreceptor outer membranes is regulated by the calcium/inositolphosphate second messenger system (5,6,7). Generation of inositolphosphates represents the “on” signal (6) whereas an increase of the cytosolic free calcium represents the “off” signal for phagocytosis (5). The RPE cells from RCS rats are able to bind shed photoreceptor membranes but are unable to ingest these membranes (1,2,3,4). Thus, RPE cells express receptors for photoreceptor membranes but the second messenger system seems to be unable to initialize the ingestion of bound membranes (7). Several lines of evidence point to a altered second messenger metabolism in RPE cells of RCS rats. Abnormal inositol phosphate generation (8), a reduced cAMP generation (9), changed protein phosphorylation (10) and, in consequence, a changed growth factor responsiveness have been reported (11,12).

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© 1997 Springer Science+Business Media New York

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Strauß, O., Mergler, S., Wienrich, M., Wiederholt, M. (1997). Altered Regulation of Ion Channels in Cultured Retinal Pigment Epithelial Cells From Rcs Rats. In: LaVail, M.M., Hollyfield, J.G., Anderson, R.E. (eds) Degenerative Retinal Diseases. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-5933-7_13

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  • DOI: https://doi.org/10.1007/978-1-4615-5933-7_13

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4613-7718-4

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