ATF6 is required for efficient rhodopsin clearance and retinal homeostasis in the P23H rho retinitis pigmentosa mouse model

Retinitis Pigmentosa (RP) is a blinding disease that arises from loss of rods and subsequently cones. The P23H rhodopsin knock-in (P23H-KI) mouse develops retinal degeneration that mirrors RP phenotype in patients carrying the orthologous variant. Previously, we found that the P23H rhodopsin protein...

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Veröffentlicht in:	Scientific reports Jg. 11; H. 1; S. 16356 - 14
Hauptverfasser:	Lee, Eun-Jin, Chan, Priscilla, Chea, Leon, Kim, Kyle, Kaufman, Randal J., Lin, Jonathan H.
Format:	Journal Article
Sprache:	Englisch
Veröffentlicht:	London Nature Publishing Group UK 11.08.2021 Nature Publishing Group Nature Portfolio
Schlagworte:	631/45 692/4017 692/420 Activating transcription factor 6 Activating Transcription Factor 6 - metabolism Animals Cones Disease Models, Animal Female Homeostasis Homeostasis - physiology Humanities and Social Sciences Male Mice Mice, Inbred C57BL multidisciplinary Phenotypes Photoreceptors Protein folding Proteins Retina Retina - metabolism Retinal degeneration Retinal Degeneration - metabolism Retinal Rod Photoreceptor Cells - metabolism Retinitis Retinitis pigmentosa Retinitis Pigmentosa - metabolism Rhodopsin Rhodopsin - metabolism Science Science (multidisciplinary)
ISSN:	2045-2322, 2045-2322
Online-Zugang:	Volltext
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