Pathogenic mutation of UBQLN2 impairs its interaction with UBXD8 and disrupts endoplasmic reticulum-associated protein degradation

Expression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in rats

(2013) Jianbin Tong et al.   EMBO JOURNAL

A Ubiquitin-like Domain Recruits an Oligomeric Chaperone to a Retrotranslocation Complex in Endoplasmic Reticulum-associated Degradation

(2013) Yue Xu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Reactive astrocytes secrete lcn2 to promote neuron death

(2013) F. Bi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Entorhinal cortical neurons are the primary targets of FUS mislocalization and ubiquitin aggregation in FUS transgenic rats

(2012) Cao Huang et al.   HUMAN MOLECULAR GENETICS

Sustained Expression of TDP-43 and FUS in Motor Neurons in Rodent's Lifetime

(2012) Cao Huang et al.   International Journal of Biological Sciences

TDP-43 Potentiates Alpha-synuclein Toxicity to Dopaminergic Neurons in Transgenic Mice

(2012) Tian Tian et al.   International Journal of Biological Sciences

Neurotoxin-induced ER stress in mouse dopaminergic neurons involves downregulation of TRPC1 and inhibition of AKT/mTOR signaling

(2012) Senthil Selvaraj et al.   JOURNAL OF CLINICAL INVESTIGATION

XBP1 depletion precedes ubiquitin aggregation and Golgi fragmentation in TDP-43 transgenic rats

(2012) Jianbin Tong et al.   JOURNAL OF NEUROCHEMISTRY

Reduced Calreticulin Levels Link Endoplasmic Reticulum Stress and Fas-Triggered Cell Death in Motoneurons Vulnerable to ALS

(2012) N. Bernard-Marissal et al.   JOURNAL OF NEUROSCIENCE

Dynamic flexibility of the ATPase p97 is important for its interprotomer motion transmission

(2012) C. Huang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Importin β Interacts with the Endoplasmic Reticulum-associated Degradation Machinery and Promotes Ubiquitination and Degradation of Mutant α1-Antitrypsin

(2011) Yongwang Zhong et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats

(2011) Cao Huang et al.   JOURNAL OF CLINICAL INVESTIGATION

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

FUS Transgenic Rats Develop the Phenotypes of Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration

(2011) Cao Huang et al.   PLoS Genetics

Transgenic Rat Model of Neurodegeneration Caused by Mutation in the TDP Gene

(2010) Hongxia Zhou et al.   PLoS Genetics

Ubiquilin and p97/VCP bind erasin, forming a complex involved in ERAD

(2009) Precious J. Lim et al.   JOURNAL OF CELL BIOLOGY

Seipinopathy: a novel endoplasmic reticulum stress-associated disease

(2008) Daisuke Ito et al.   BRAIN

ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1

(2008) H. Nishitoh et al.   GENES & DEVELOPMENT

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

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

Unsaturated Fatty Acids Inhibit Proteasomal Degradation of Insig-1 at a Postubiquitination Step

(2008) Joon No Lee et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

SEL1L nucleates a protein complex required for dislocation of misfolded glycoproteins

(2008) B. Mueller et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA