Induction of STAT3-related genes in fast degenerating cone photoreceptors of cpfl1 mice
| Autor: | M. W. Seeliger, Bernd Wissinger, Karin Schaeferhoff, Stylianos Michalakis, Gesine Huber, Naoyuki Tanimoto, M. D. Fischer, Olaf Riess, Susanne C. Beck, Elvir Becirovic, N. Rieger, Michael Bonin, Martin Biel |
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| Rok vydání: | 2010 |
| Předmět: |
Retinal degeneration
STAT3 Transcription Factor Pathology medicine.medical_specialty genetic structures Mutant Degeneration (medical) Biology medicine.disease_cause Cellular and Molecular Neuroscience Mice medicine Animals STAT3 Molecular Biology Loss function Oligonucleotide Array Sequence Analysis Pharmacology Mutation medicine.diagnostic_test Retinal Degeneration Cell Biology medicine.disease Mice Mutant Strains Cell biology Gliosis Gene Expression Regulation biology.protein Retinal Cone Photoreceptor Cells Molecular Medicine sense organs medicine.symptom Electroretinography |
| Zdroj: | Cellular and molecular life sciences : CMLS. 67(18) |
| ISSN: | 1420-9071 |
| Popis: | Cone dystrophies are genetic diseases characterized by loss of cone photoreceptor function and severe impairment of daylight vision. Loss of function is accompanied by a progressive degeneration of cones limiting potential therapeutic interventions. In this study we combined microarray-based gene-expression analysis with electroretinography and immunohistochemistry to characterize the pathological processes in the cone photoreceptor function loss 1 (cpfl1) mouse model. The cpfl1-mouse is a naturally arising mouse mutant with a loss-of-function mutation in the cone-specific Pde6c gene. Cpfl1-mice displayed normal rod-specific light responses while cone-specific responses were strongly diminished. Despite the lack of a general retinal degeneration, the cone-specific functional defect resulted in a marked activation of GFAP, a hallmark of Muller-cell gliosis. Microarray-based network-analysis confirmed activation of Muller-glia-specific transcripts. Unexpectedly, we found up-regulation of the cytokine LIF and the anti-apoptotic transcription factor STAT3 in cpfl1 cone photoreceptors. We postulate that STAT3-related pathways are induced in cpfl1 cone photoreceptors to counteract degeneration. |
| Databáze: | OpenAIRE |
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