Early apoptosis of rod photoreceptors in Rpe65−/− mice is associated with the upregulated expression of lysosomal-mediated autophagic genes

RPE65-related Leber's congenital amaurosis (LCA) is a rod-cone dystrophy whose clinical outcome is mainly attributed to the loss of rod photoreceptors followed by cone degeneration. Pathogenesis in Rpe65−/− mice is characterized by a slow and progressive degeneration of rods dependent on the co...

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Veröffentlicht in:	Experimental eye research 2012-03, Vol.96 (1), p.70-81
Hauptverfasser:	Métrailler, Sylviane, Schorderet, Daniel F., Cottet, Sandra
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Apoptosis Autophagy Bax protein bcl-2-Associated X Protein - genetics Blindness Blotting, Western Carrier Proteins - genetics Caspase Cathepsin S Cathepsins - genetics Cell fate cis-trans-Isomerases Cones Degeneration Dystrophy Eye Eye Proteins - genetics Fluorescent Antibody Technique, Indirect Gene Expression Regulation - physiology Genotyping Techniques hydrolase In Situ Nick-End Labeling LAMP-2 protein Leber Congenital Amaurosis - genetics Leber Congenital Amaurosis - pathology Lysosomal-Associated Membrane Protein 2 - genetics Lysozyme Mice Mice, Inbred BALB C Mice, Inbred C57BL Mice, Knockout Muramidase - genetics opsins Phagocytosis Photoreceptors Phototransduction Proto-Oncogene Proteins c-bcl-2 - genetics Real-Time Polymerase Chain Reaction Retina Retinal Rod Photoreceptor Cells - pathology rod Rods Signal transduction Stress survival Waves
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