Contribution of the Unfolded Protein Response (UPR) to the Pathogenesis of Proteasome-Associated Autoinflammatory Syndromes (PRAAS)

Contribution of the Unfolded Protein Response (UPR) to the Pathogenesis of Proteasome-Associated Autoinflammatory Syndromes (PRAAS)

Type I interferonopathies cover a phenotypically heterogeneous group of rare genetic diseases including the recently described proteasome-associated autoinflammatory syndromes (PRAAS). By definition, PRAAS are caused by inherited and/or de novo loss-of-function mutations in genes encoding proteasome...

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Bibliographic Details
Main Authors: Frédéric Ebstein, María Cecilia Poli Harlowe, Maja Studencka-Turski, Elke Krüger
Format: Article
Language:English
Published: Frontiers Media S.A. 2019-11-01
Series:Frontiers in Immunology
Subjects:
proteasome
ER stress
unfolded protein response
mTORC1
TCF11/Nrf1
autoinflammation
Online Access:https://www.frontiersin.org/article/10.3389/fimmu.2019.02756/full

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https://www.frontiersin.org/article/10.3389/fimmu.2019.02756/full

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