Selective activation of ATF6 and PERK endoplasmic reticulum stress signaling pathways prevent mutant rhodopsin accumulation
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Endoplasmic reticulum quality control and systemic amyloid disease: Impacting protein stability from the inside outThe effects of IRE1, ATF6, and PERK signaling on adRP-linked rhodopsinsPhotoactivation-induced instability of rhodopsin mutants T4K and T17M in rod outer segments underlies retinal degeneration in X. laevis transgenic models of retinitis pigmentosaUnfolded protein response activation reduces secretion and extracellular aggregation of amyloidogenic immunoglobulin light chainATF6 activation reduces the secretion and extracellular aggregation of destabilized variants of an amyloidogenic protein.Chemical chaperone 4-phenylbutyrate prevents endoplasmic reticulum stress induced by T17M rhodopsin.ATF6 Is Mutated in Early Onset Photoreceptor Degeneration With Macular Involvement.Mutation of ATF6 causes autosomal recessive achromatopsia.Involvement of Endoplasmic Reticulum Stress in TULP1 Induced Retinal Degeneration.Correlation of ER stress and retinal degeneration in tubby mice.In Vivo Visualization of Endoplasmic Reticulum Stress in the Retina Using the ERAI Reporter MouseLight Induces Ultrastructural Changes in Rod Outer and Inner Segments, Including Autophagy, in a Transgenic Xenopus laevis P23H Rhodopsin Model of Retinitis Pigmentosa.Induction of endoplasmic reticulum stress genes, BiP and chop, in genetic and environmental models of retinal degenerationStress-independent activation of XBP1s and/or ATF6 reveals three functionally diverse ER proteostasis environmentsReview: retinal degeneration: focus on the unfolded protein response.Achromatopsia mutations target sequential steps of ATF6 activation.Translational and posttranslational regulation of XIAP by eIF2α and ATF4 promotes ER stress-induced cell death during the unfolded protein response.Targeting unfolded protein response signaling pathways to ameliorate protein misfolding diseases.The cell stress machinery and retinal degeneration.Small molecule proteostasis regulators that reprogram the ER to reduce extracellular protein aggregation.Multiple Mechanisms of Unfolded Protein Response-Induced Cell DeathAAV Delivery of GRP78/BiP Promotes Adaptation of Human RPE Cell to ER Stress.The Role of Endoplasmic Reticulum Stress in Diabetic Nephropathy.Regulating Secretory Proteostasis through the Unfolded Protein Response: From Function to Therapy.Mouse models of human ocular disease for translational research.Endoplasmic reticulum stress in vertebrate mutant rhodopsin models of retinal degeneration.Mechanisms of L-Serine Neuroprotection in vitro Include ER Proteostasis Regulation.Adapting Secretory Proteostasis and Function Through the Unfolded Protein Response.Endoplasmic reticulum (ER) Ca2+-channel activity contributes to ER stress and cone death in cyclic nucleotide-gated channel deficiency.
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P2860
Selective activation of ATF6 and PERK endoplasmic reticulum stress signaling pathways prevent mutant rhodopsin accumulation
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2012 nî lūn-bûn
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2012年の論文
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2012年学术文章
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2012年学术文章
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2012年学术文章
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2012年学术文章
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2012年學術文章
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name
Selective activation of ATF6 a ...... mutant rhodopsin accumulation
@ast
Selective activation of ATF6 a ...... mutant rhodopsin accumulation
@en
type
label
Selective activation of ATF6 a ...... mutant rhodopsin accumulation
@ast
Selective activation of ATF6 a ...... mutant rhodopsin accumulation
@en
prefLabel
Selective activation of ATF6 a ...... mutant rhodopsin accumulation
@ast
Selective activation of ATF6 a ...... mutant rhodopsin accumulation
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P2093
P2860
P356
P1476
Selective activation of ATF6 a ...... mutant rhodopsin accumulation
@en
P2093
Carissa Messah
Heike Kroeger
Jonathan H Lin
Nobuhiko Hiramatsu
P2860
P304
P356
10.1167/IOVS.12-10222
P407
P577
2012-10-01T00:00:00Z