title: The role of caspases in photoreceptor cell death of the retinoschisin-deficient mouse.
abstract: Early schisis cavities in the retinal bipolar cell layer accompanied by progressive loss of cone and rod photoreceptor cells are the hallmark of the retinoschisin-deficient (Rs1h(-/Y)) murine retina. With this study we aimed at elucidating the molecular events underlying the photoreceptor cell death in this established murine model of X-linked juvenile retinoschisis. We show that photoreceptor degeneration in the Rs1h(-/Y) mouse is due to apoptotic events peaking around postnatal day 18. Cell death is accompanied by increased expression of initiator and inflammatory caspases but not by downstream effector caspases. The strong induction of caspase-1 (Casp1) prompted us to explore its involvement in the apoptotic process. We therefore generated double knock-out mice deficient for both retinoschisin and Casp1. No direct influence of the Casp1 genotype on apoptosis could be identified although striking differences in the overall number of resident microglia were observed independent of the Rs1h genotype.
authors.affiliation: Institute of Human Genetics, University of Regensburg, Regensburg, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Gehrig
authors.forename: A
authors.initials: A
authors.lastname: Janssen
authors.forename: F
authors.initials: F
authors.lastname: Horling
authors.forename: C
authors.initials: C
authors.lastname: Grimm
authors.forename: B H F
authors.initials: BH
authors.lastname: Weber
mesh_terms: D000818:Animals; D017209:Apoptosis; D020170:Caspase 1 / Q000235:genetics / Q000502:physiology*; D020169:Caspases / Q000235:genetics / Q000502:physiology*; D015815:Cell Adhesion Molecules / Q000235:genetics*; D002452:Cell Count; D005136:Eye Proteins / Q000235:genetics*; D015971:Gene Expression Regulation, Enzymologic; D005838:Genotype; D051379:Mice; D017628:Microglia; D020419:Photoreceptor Cells, Vertebrate; D041441:Retinoschisis / Q000235:genetics / Q000473:pathology
chemical_list: D015815:Cell Adhesion Molecules; D005136:Eye Proteins; C491843:RS1 protein, mouse; D020169:Caspases; D020170:Caspase 1