Ubiquilin-2 drives NF-κB activity and cytosolic TDP-43 aggregation in neuronal cells

Viral expression of ALS-linked ubiquilin-2 mutants causes inclusion pathology and behavioral deficits in mice

(2015) Carolina Ceballos-Diaz et al.   Molecular Neurodegeneration

Pathogenic Ubqln2 gains toxic properties to induce neuron death

(2014) Qinxue Wu et al.   ACTA NEUROPATHOLOGICA

Ubiquilin 2: A component of the ubiquitin–proteasome system with an emerging role in neurodegeneration

(2014) Katharine Y. Zhang et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Endoplasmic reticulum stress is accompanied by activation of NF-κB in amyotrophic lateral sclerosis

(2014) T. Prell et al.   JOURNAL OF NEUROIMMUNOLOGY

Tumor Necrosis Factor Alpha: A Link between Neuroinflammation and Excitotoxicity

(2014) Gabriel Olmos et al.   MEDIATORS OF INFLAMMATION

Microglia Induce Motor Neuron Death via the Classical NF-κB Pathway in Amyotrophic Lateral Sclerosis

(2014) Ashley E. Frakes et al.   NEURON

Early-Stage Treatment with Withaferin A Reduces Levels of Misfolded Superoxide Dismutase 1 and Extends Lifespan in a Mouse Model of Amyotrophic Lateral Sclerosis

(2014) Priyanka Patel et al.   Neurotherapeutics

Dendritic spinopathy in transgenic mice expressing ALS/dementia-linked mutantUBQLN2

(2014) George H. Gorrie et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Advances in treating amyotrophic lateral sclerosis: insights from pathophysiological studies

(2014) Steve Vucic et al.   TRENDS IN NEUROSCIENCES

Protein aggregation in amyotrophic lateral sclerosis

(2013) Anna M. Blokhuis et al.   ACTA NEUROPATHOLOGICA

Unbiased screen reveals ubiquilin-1 and -2 highly associated with huntingtin inclusions

(2013) Nicola J. Rutherford et al.   BRAIN RESEARCH

Molecular mechanism of oxidation-induced TDP-43 RRM1 aggregation and loss of function

(2013) Chung-ke Chang et al.   FEBS LETTERS

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

(2013) Yuxing Xia et al.   JOURNAL OF NEUROCHEMISTRY

Optineurin suppression causes neuronal cell death via NF-κB pathway

(2013) Mayumi Akizuki et al.   JOURNAL OF NEUROCHEMISTRY

Ubiquilin 2mutations in Italian patients with amyotrophic lateral sclerosis and frontotemporal dementia

(2012) Cinzia Gellera et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Screening in ALS and FTD patients reveals 3 novel UBQLN2 mutations outside the PXX domain and a pure FTD phenotype

(2012) Matthis Synofzik et al.   NEUROBIOLOGY OF AGING

Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB–mediated pathogenic pathways

(2011) Vivek Swarup et al.   JOURNAL OF EXPERIMENTAL MEDICINE

C-Jun N-terminal kinase controls TDP-43 accumulation in stress granules induced by oxidative stress

(2011) Jodi Meyerowitz et al.   Molecular Neurodegeneration

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

(2011) Han-Xiang Deng et al.   NATURE

Oxidative stress induced by glutathione depletion reproduces pathological modifications of TDP-43 linked to TDP-43 proteinopathies

(2011) Yohei Iguchi et al.   NEUROBIOLOGY OF DISEASE

Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone

(2010) Sara K. Custer et al.   HUMAN MOLECULAR GENETICS

TNF-α potentiates glutamate-induced spinal cord motoneuron death via NF-κB

(2010) Laia Tolosa et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

VCP Associated Inclusion Body Myopathy and Paget Disease of Bone Knock-In Mouse Model Exhibits Tissue Pathology Typical of Human Disease

(2010) Mallikarjun Badadani et al.   PLoS One

Regulation and Function of NF-κB Transcription Factors in the Immune System

(2009) Sivakumar Vallabhapurapu et al.   Annual Review of Immunology

TDP-43 Redistribution is an Early Event in Sporadic Amyotrophic Lateral Sclerosis

(2009) Maria Teresa Giordana et al.   BRAIN PATHOLOGY

Oxidative stress in ALS: Key role in motor neuron injury and therapeutic target

(2009) Siân C. Barber et al.   FREE RADICAL BIOLOGY AND MEDICINE

Exaggerated inflammation, impaired host defense, and neuropathology in progranulin-deficient mice

(2009) Fangfang Yin et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Withaferin A inhibits iNOS expression and nitric oxide production by Akt inactivation and down-regulating LPS-induced activity of NF-κB in RAW 264.7 cells

(2008) Jung Hwa Oh et al.   EUROPEAN JOURNAL OF PHARMACOLOGY

A Limited Role for Disulfide Cross-linking in the Aggregation of Mutant SOD1 Linked to Familial Amyotrophic Lateral Sclerosis

(2008) Celeste M. Karch et al.   JOURNAL OF BIOLOGICAL CHEMISTRY