Marinesco-Sjögren syndrome protein SIL1 regulates motor neuron subtype-selective ER stress in ALS

How does protein misfolding in the endoplasmic reticulum affect lipid metabolism in the liver?

(2014) Shiyu Wang et al.   CURRENT OPINION IN LIPIDOLOGY

Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis

(2014) Janel O Johnson et al.   NATURE NEUROSCIENCE

Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases

(2014) Claudio Hetz et al.   NATURE REVIEWS NEUROSCIENCE

Neuronal Matrix Metalloproteinase-9 Is a Determinant of Selective Neurodegeneration

(2014) Artem Kaplan et al.   NEURON

Myopathy in Marinesco–Sjögren syndrome links endoplasmic reticulum chaperone dysfunction to nuclear envelope pathology

(2013) Andreas Roos et al.   ACTA NEUROPATHOLOGICA

SIL1 mutations and clinical spectrum in patients with Marinesco-Sjögren syndrome

(2013) Michael Krieger et al.   BRAIN

Intracerebroventricular Injection of Adeno-Associated Virus 6 and 9 Vectors for Cell Type–Specific Transgene Expression in the Spinal Cord

(2013) Elisabeth Dirren et al.   HUMAN GENE THERAPY

Altered localization, abnormal modification and loss of function of Sigma receptor-1 in amyotrophic lateral sclerosis

(2013) J. Prause et al.   HUMAN MOLECULAR GENETICS

Unfolded Protein Response Signaling and Metabolic Diseases

(2013) Jaemin Lee et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

SOD1 as a Molecular Switch for Initiating the Homeostatic ER Stress Response under Zinc Deficiency

(2013) Kengo Homma et al.   MOLECULAR CELL

Neuroprotection through Excitability and mTOR Required in ALS Motoneurons to Delay Disease and Extend Survival

(2013) Smita Saxena et al.   NEURON

ALS-Associated TDP-43 Induces Endoplasmic Reticulum Stress, Which Drives Cytoplasmic TDP-43 Accumulation and Stress Granule Formation

(2013) Adam K. Walker et al.   PLoS One

Proteome remodelling during development from blood to insect-form Trypanosoma brucei quantified by SILAC and mass spectrometry

(2012) Kapila Gunasekera et al.   BMC GENOMICS

The impact of the unfolded protein response on human disease

(2012) Shiyu Wang et al.   JOURNAL OF CELL BIOLOGY

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

Glucose Regulated Protein 78 Diminishes α-Synuclein Neurotoxicity in a Rat Model of Parkinson Disease

(2012) Marina S Gorbatyuk et al.   MOLECULAR THERAPY

Mutant FUS induces endoplasmic reticulum stress in amyotrophic lateral sclerosis and interacts with protein disulfide-isomerase

(2012) Manal A. Farg et al.   NEUROBIOLOGY OF AGING

Cause or Effect of Arteriogenesis: Compositional Alterations of Microparticles from CAD Patients Undergoing External Counterpulsation Therapy

(2012) Ali Al Kaabi et al.   PLoS One

Structural analysis of the Sil1–Bip complex reveals the mechanism for Sil1 to function as a nucleotide-exchange factor

(2011) Ming Yan et al.   BIOCHEMICAL JOURNAL

Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders

(2011) Karen M. Doyle et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Selective Neuronal Vulnerability in Neurodegenerative Diseases: from Stressor Thresholds to Degeneration

(2011) Smita Saxena et al.   NEURON

Excitation–transcription coupling in sympathetic neurons and the molecular mechanism of its initiation

(2011) Huan Ma et al.   NEUROSCIENCE RESEARCH

The Unfolded Protein Response: Integrating Stress Signals Through the Stress Sensor IRE1α

(2011) Claudio Hetz et al.   PHYSIOLOGICAL REVIEWS

The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation

(2011) P. Walter et al.   SCIENCE

Motor Neuron Diversity in Development and Disease

(2010) Kevin C. Kanning et al.   Annual Review of Neuroscience

Endoplasmic reticulum stress-sensing mechanisms in yeast and mammalian cells

(2010) Yukio Kimata et al.   CURRENT OPINION IN CELL BIOLOGY

Chromosome 9p21 in sporadic amyotrophic lateral sclerosis in the UK and seven other countries: a genome-wide association study

(2010) Aleksey Shatunov et al.   LANCET NEUROLOGY

Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS

(2010) Janel O. Johnson et al.   NEURON

Modification of Superoxide Dismutase 1 (SOD1) Properties by a GFP Tag – Implications for Research into Amyotrophic Lateral Sclerosis (ALS)

(2010) James C. Stevens et al.   PLoS One

Autosomal-Dominant Distal Myopathy Associated with a Recurrent Missense Mutation in the Gene Encoding the Nuclear Matrix Protein, Matrin 3

(2009) Jan Senderek et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Upregulation of persistent sodium conductances in familial ALS

(2009) S. Vucic et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

A role for motoneuron subtype–selective ER stress in disease manifestations of FALS mice

(2009) Smita Saxena et al.   NATURE NEUROSCIENCE

TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration

(2009) I. Wegorzewska et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

An ALS-Linked Mutant SOD1 Produces a Locomotor Defect Associated with Aggregation and Synaptic Dysfunction When Expressed in Neurons of Caenorhabditis elegans

(2009) Jiou Wang et al.   PLoS Genetics

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

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

Preferential motor unit loss in the SOD1G93Atransgenic mouse model of amyotrophic lateral sclerosis

(2008) J. Hegedus et al.   JOURNAL OF PHYSIOLOGY-LONDON

Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis

(2008) Julie D. Atkin et al.   NEUROBIOLOGY OF DISEASE

Integrating the stress response: lessons for neurodegenerative diseases from C. elegans

(2008) Veena Prahlad et al.   TRENDS IN CELL BIOLOGY