ER Stress-Induced eIF2-alpha Phosphorylation Underlies Sensitivity of Striatal Neurons to Pathogenic Huntingtin

ER-stress-induced transcriptional regulation increases protein synthesis leading to cell death

(2013) Jaeseok Han et al.   NATURE CELL BIOLOGY

Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress

(2013) Julia Leitman et al.   Nature Communications

Pharmacological brake-release of mRNA translation enhances cognitive memory

(2013) Carmela Sidrauski et al.   eLife

Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy

(2012) Rene L. Vidal et al.   HUMAN MOLECULAR GENETICS

Indirect inhibition of 26S proteasome activity in a cellular model of Huntington’s disease

(2012) Mark S. Hipp et al.   JOURNAL OF CELL BIOLOGY

Endoplasmic reticulum dysfunction in neurological disease

(2012) Benoit D Roussel et al.   LANCET NEUROLOGY

Perk-dependent repression of miR-106b-25 cluster is required for ER stress-induced apoptosis

(2012) S Gupta et al.   Cell Death & Disease

Converging Pathways in the Occurrence of Endoplasmic Reticulum (ER) Stress in Huntingtons Disease

(2011) R. Vidal et al.   CURRENT MOLECULAR MEDICINE

IRE1 plays an essential role in ER stress-mediated aggregation of mutant huntingtin via the inhibition of autophagy flux

(2011) Huikyong Lee et al.   HUMAN MOLECULAR GENETICS

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

Bypass of glycan-dependent glycoprotein delivery to ERAD by up-regulated EDEM1

(2011) Efrat Ron et al.   MOLECULAR BIOLOGY OF THE CELL

Activation of PKR Causes Amyloid ß-Peptide Accumulation via De-Repression of BACE1 Expression

(2011) Gerard ILL-Raga et al.   PLoS One

Selective Inhibition of a Regulatory Subunit of Protein Phosphatase 1 Restores Proteostasis

(2011) P. Tsaytler et al.   SCIENCE

The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation

(2011) P. Walter et al.   SCIENCE

Huntington’s disease and striatal signaling

(2011) Emmanuel Roze   Frontiers in Neuroanatomy

Structural Determinants of PERK Inhibitor Potency and Selectivity

(2010) Hong Wang et al.   Chemical Biology & Drug Design

Neurocognitive signs in prodromal Huntington disease.

(2010) Julie C. Stout et al.   NEUROPSYCHOLOGY

Huntingtin Interacts with the Cue Domain of gp78 and Inhibits gp78 Binding to Ubiquitin and p97/VCP

(2010) Hui Yang et al.   PLoS One

Rrs1 Is Involved in Endoplasmic Reticulum Stress Response in Huntington Disease

(2009) Alisia Carnemolla et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Rhes, a Striatal Specific Protein, Mediates Mutant-Huntingtin Cytotoxicity

(2009) S. Subramaniam et al.   SCIENCE

Huntington's disease: from pathology and genetics to potential therapies

(2008) Sara Imarisio et al.   BIOCHEMICAL JOURNAL

Exendin-4 Improves Glycemic Control, Ameliorates Brain and Pancreatic Pathologies, and Extends Survival in a Mouse Model of Huntington's Disease

(2008) B. Martin et al.   DIABETES

Inhibition of endoplasmic reticulum stress counteracts neuronal cell death and protein aggregation caused by N-terminal mutant huntingtin proteins

(2008) Sami Reijonen et al.   EXPERIMENTAL CELL RESEARCH

Impaired ERAD and ER stress are early and specific events in polyglutamine toxicity

(2008) M. L. Duennwald et al.   GENES & DEVELOPMENT

The Ubiquitin-Proteasome Pathway in Huntington's Disease

(2008) Siddhartha Mitra et al.   TheScientificWorldJOURNAL