Familial prion protein mutants inhibit Hrd1-mediated retrotranslocation of misfolded proteins by depleting misfolded protein sensor BiP

ER Stress-Induced Clearance of Misfolded GPI-Anchored Proteins via the Secretory Pathway

(2014) Prasanna Satpute-Krishnan et al.   CELL

ER-associated degradation: Protein quality control and beyond

(2014) Annamaria Ruggiano et al.   JOURNAL OF CELL BIOLOGY

Druggable sensors of the unfolded protein response

(2014) Dustin J Maly et al.   Nature Chemical Biology

Ubiquitin Ligase gp78 Targets Unglycosylated Prion Protein PrP for Ubiquitylation and Degradation

(2014) Jia Shao et al.   PLoS One

Sel1L is indispensable for mammalian endoplasmic reticulum-associated degradation, endoplasmic reticulum homeostasis, and survival

(2014) Shengyi Sun et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Endoplasmic reticulum lectin XTP3-B inhibits endoplasmic reticulum-associated degradation of a misfolded α1-antitrypsin variant

(2013) Tsutomu Fujimori et al.   FEBS Journal

Der1 promotes movement of misfolded proteins through the endoplasmic reticulum membrane

(2013) Martin Mehnert et al.   NATURE CELL BIOLOGY

Snord 3A: A Molecular Marker and Modulator of Prion Disease Progression

(2013) Eran Cohen et al.   PLoS One

Oral Treatment Targeting the Unfolded Protein Response Prevents Neurodegeneration and Clinical Disease in Prion-Infected Mice

(2013) J. A. Moreno et al.   Science Translational Medicine

A Shared Endoplasmic Reticulum-associated Degradation Pathway Involving the EDEM1 Protein for Glycosylated and Nonglycosylated Proteins

(2012) Marina Shenkman et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Sustained translational repression by eIF2α-P mediates prion neurodegeneration

(2012) Julie A. Moreno et al.   NATURE

The unfolded protein response: controlling cell fate decisions under ER stress and beyond

(2012) Claudio Hetz   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Changes in BiP availability reveal hypersensitivity to acute endoplasmic reticulum stress in cells expressing mutant huntingtin

(2011) P. Lajoie et al.   JOURNAL OF CELL SCIENCE

Defining human ERAD networks through an integrative mapping strategy

(2011) John C. Christianson et al.   NATURE CELL BIOLOGY

Familial CJD Associated PrP Mutants within Transmembrane Region Induced Ctm-PrP Retention in ER and Triggered Apoptosis by ER Stress in SH-SY5Y Cells

(2011) Xin Wang et al.   PLoS One

Expression of Mutant or Cytosolic PrP in Transgenic Mice and Cells Is Not Associated with Endoplasmic Reticulum Stress or Proteasome Dysfunction

(2011) Elena Quaglio et al.   PLoS One

Efficient Detection of Proteins Retro-Translocated from the ER to the Cytosol by In Vivo Biotinylation

(2011) Gianluca Petris et al.   PLoS One

Loss of HRD1-Mediated Protein Degradation Causes Amyloid Precursor Protein Accumulation and Amyloid-  Generation

(2010) M. Kaneko et al.   JOURNAL OF NEUROSCIENCE

Yokukansan Inhibits Neuronal Death during ER Stress by Regulating the Unfolded Protein Response

(2010) Toru Hiratsuka et al.   PLoS One

The Endoplasmic Reticulum-associated Degradation of Transthyretin Variants Is Negatively Regulated by BiP in Mammalian Cells

(2009) Seiko Susuki et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

N-Glycosylation Enhances Presentation of a MHC Class I-Restricted Epitope from Tyrosinase

(2009) M. Ostankovitch et al.   JOURNAL OF IMMUNOLOGY

Loss of Anti-Bax Function in Gerstmann-Sträussler-Scheinker Syndrome-Associated Prion Protein Mutants

(2009) Julie Jodoin et al.   PLoS One

Role of GRP78/BiP Degradation and ER Stress in Deoxynivalenol-Induced Interleukin-6 Upregulation in the Macrophage

(2009) Yuhui Shi et al.   TOXICOLOGICAL SCIENCES

Selective Processing and Metabolism of Disease-Causing Mutant Prion Proteins

(2009) Aarthi Ashok et al.   PLoS Pathogens

Cytosolic prion protein is the predominant anti-Bax prion protein form: Exclusion of transmembrane and secreted prion protein forms in the anti-Bax function

(2008) David T.S. Lin et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

A randomized cross-over study of a traditional Japanese medicine (kampo), yokukansan, in the treatment of the behavioural and psychological symptoms of dementia

(2008) Katsuyoshi Mizukami et al.   INTERNATIONAL JOURNAL OF NEUROPSYCHOPHARMACOLOGY

OS-9 and GRP94 deliver mutant α1-antitrypsin to the Hrd1–SEL1L ubiquitin ligase complex for ERAD

(2008) John C. Christianson et al.   NATURE CELL BIOLOGY

Unfolded protein response transcription factor XBP-1 does not influence prion replication or pathogenesis

(2008) C. Hetz et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA