Ubiquitin-specific protease-14 reduces cellular aggregates and protects against mutant huntingtin-induced cell degeneration: involvement of the proteasome and ER stress-activated kinase IRE1 

Hypothalamic expression of mutant huntingtin contributes to the development of depressive-like behavior in the BAC transgenic mouse model of Huntington's disease

(2013) Sofia Hult Lundh et al.   HUMAN MOLECULAR GENETICS

Assessment of Motor Function, Sensory Motor Gating and Recognition Memory in a Novel BACHD Transgenic Rat Model for Huntington Disease

(2013) Yah-se K. Abada et al.   PLoS One

Sigma-1 receptor agonist PRE084 is protective against mutant huntingtin-induced cell degeneration: involvement of calpastatin and the NF-κB pathway

(2013) A Hyrskyluoto et al.   Cell Death & Disease

Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice

(2012) M. A. Pouladi et al.   HUMAN MOLECULAR GENETICS

Fibroblast Growth Factor-21 (FGF21) Regulates Low-density Lipoprotein Receptor (LDLR) Levels in Cells via the E3-ubiquitin Ligase Mylip/Idol and the Canopy2 (Cnpy2)/Mylip-interacting Saposin-like Protein (Msap)

(2012) Hai Thi Do et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

GADD34 mediates cytoprotective autophagy in mutant huntingtin expressing cells via the mTOR pathway

(2011) Alise Hyrskyluoto et al.   EXPERIMENTAL CELL RESEARCH

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

Clearance of Mutant Proteins as a Therapeutic Target in Neurodegenerative Diseases

(2010) Dimitri Krainc   ARCHIVES OF NEUROLOGY

Downregulation of NF-κB signaling by mutant huntingtin proteins induces oxidative stress and cell death

(2010) Sami Reijonen et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

The regulation of autophagy - unanswered questions

(2010) Y. Chen et al.   JOURNAL OF CELL SCIENCE

Huntington's disease: from molecular pathogenesis to clinical treatment

(2010) Christopher A Ross et al.   LANCET NEUROLOGY

The Selective Macroautophagic Degradation of Aggregated Proteins Requires the PI3P-Binding Protein Alfy

(2010) Maria Filimonenko et al.   MOLECULAR CELL

Network organization of the human autophagy system

(2010) Christian Behrends et al.   NATURE

Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease

(2010) Chiara Zuccato et al.   PHYSIOLOGICAL REVIEWS

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

(2010) Hui Yang et al.   PLoS One

USP14 inhibits ER-associated degradation via interaction with IRE1α

(2009) Atsushi Nagai et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Mechanisms of neurodegeneration in Huntington’s disease

(2008) Joana M. Gil et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

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

Full-Length Human Mutant Huntingtin with a Stable Polyglutamine Repeat Can Elicit Progressive and Selective Neuropathogenesis in BACHD Mice

(2008) M. Gray et al.   JOURNAL OF NEUROSCIENCE

Relative Structural and Functional Roles of Multiple Deubiquitylating Proteins Associated with Mammalian 26S Proteasome

(2007) Elena Koulich et al.   MOLECULAR BIOLOGY OF THE CELL