Achromatopsia mutations target sequential steps of ATF6 activation. - Wikidata - wikimedia - TriplyDB

Achromatopsia mutations target sequential steps of ATF6 activation.

Achromatopsia mutations target sequential steps of ATF6 activation.

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Recent Insights into the Role of Unfolded Protein Response in ER Stress in Health and Disease.ER stress and the unfolded protein response in neurodegeneration.Gene therapy for achromatopsia.Regulating Secretory Proteostasis through the Unfolded Protein Response: From Function to Therapy.Physiological/pathological ramifications of transcription factors in the unfolded protein response.Autosomal recessive cone-rod dystrophy can be caused by mutations in the ATF6 gene.Emerging therapeutic targets currently under investigation for the treatment of systemic amyloidosis.Achromatopsia: clinical features, molecular genetics, animal models and therapeutic options.The unfolded protein response regulator ATF6 promotes mesodermal differentiation.Loss of XBP1 accelerates age-related decline in retinal function and neurodegeneration.Unfolding the Endoplasmic Reticulum of a Social Amoeba: Dictyostelium discoideum as a New Model for the Study of Endoplasmic Reticulum Stress.Pharmacologic ATF6 activating compounds are metabolically activated to selectively modify endoplasmic reticulum proteins

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Achromatopsia mutations target sequential steps of ATF6 activation.

http://www.wikidata.org/entity/Q37589879

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article científic

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article scientifique

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articolo scientifico

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artigo científico

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bilimsel makale

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scientific article published on 27 December 2016

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vedecký článok

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vetenskaplig artikel

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videnskabelig artikel

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vědecký článek

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name

Achromatopsia mutations target sequential steps of ATF6 activation.

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Achromatopsia mutations target sequential steps of ATF6 activation.

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type

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Achromatopsia mutations target sequential steps of ATF6 activation.

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Achromatopsia mutations target sequential steps of ATF6 activation.

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prefLabel

Achromatopsia mutations target sequential steps of ATF6 activation.

@en

Achromatopsia mutations target sequential steps of ATF6 activation.

@nl

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PNAS.1606387114

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Proceedings of the National Academy of Sciences of the United States of America

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Achromatopsia mutations target sequential steps of ATF6 activation

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Anna Skorczyk-Werner

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Bernd Wissinger

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Elise Héon

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Jonathan H Lin

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Priscilla Chan

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Randal J Kaufman

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Stephen H Tsang

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Mammalian transcription factor ATF6 is synthesized as a transmembrane protein and activated by proteolysis in response to endoplasmic reticulum stress

ER stress induces cleavage of membrane-bound ATF6 by the same proteases that process SREBPs

XBP1 mRNA is induced by ATF6 and spliced by IRE1 in response to ER stress to produce a highly active transcription factor

The unfolded protein response in retinal vascular diseases: implications and therapeutic potential beyond protein folding

Mutations in the unfolded protein response regulator ATF6 cause the cone dysfunction disorder achromatopsia

Perk is essential for translational regulation and cell survival during the unfolded protein response

Translational control is required for the unfolded protein response and in vivo glucose homeostasis

IRE1 couples endoplasmic reticulum load to secretory capacity by processing the XBP-1 mRNA

Regulated translation initiation controls stress-induced gene expression in mammalian cells

ER stress regulation of ATF6 localization by dissociation of BiP/GRP78 binding and unmasking of Golgi localization signals

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400-405

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scholarly article

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10.1073/PNAS.1606387114

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2

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114

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Maciej R Krawczyński

Wei-Chieh Chiang

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2016-12-27T00:00:00Z

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28028229

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5240680

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