Published in Acta Neuropathol on July 28, 2007
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Expansion of CUG RNA repeats causes stress and inhibition of translation in myotonic dystrophy 1 (DM1) cells. FASEB J (2010) 1.09
A mouse model offers novel insights into the myopathy and tendinopathy often associated with pseudoachondroplasia and multiple epiphyseal dysplasia. Hum Mol Genet (2010) 1.00
Tribbles 3 mediates endoplasmic reticulum stress-induced insulin resistance in skeletal muscle. Nat Commun (2013) 0.97
Endoplasmic reticulum stress in skeletal muscle homeostasis and disease. Curr Rheumatol Rep (2012) 0.94
Progressive impairment of muscle regeneration in muscleblind-like 3 isoform knockout mice. Hum Mol Genet (2013) 0.92
Dysferlin and animal models for dysferlinopathy. J Toxicol Pathol (2012) 0.86
Inhibition of ER stress and unfolding protein response pathways causes skeletal muscle wasting during cancer cachexia. FASEB J (2016) 0.85
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Fuchs endothelial corneal dystrophy in patients with myotonic dystrophy: a case series. Cornea (2014) 0.76
Emerging Roles of ER Stress and Unfolded Protein Response Pathways in Skeletal Muscle Health and Disease. J Cell Physiol (2017) 0.76
Sarcolipin expression is repressed by endoplasmic reticulum stress in C2C12 myotubes. J Physiol Biochem (2017) 0.75
Autophagy is activated for cell survival after endoplasmic reticulum stress. Mol Cell Biol (2006) 8.89
Genome-wide association study identifies common variants at four loci as genetic risk factors for Parkinson's disease. Nat Genet (2009) 8.11
Expanded CUG repeats trigger aberrant splicing of ClC-1 chloride channel pre-mRNA and hyperexcitability of skeletal muscle in myotonic dystrophy. Mol Cell (2002) 4.44
Involvement of caspase-4 in endoplasmic reticulum stress-induced apoptosis and Abeta-induced cell death. J Cell Biol (2004) 3.85
Targeting nuclear RNA for in vivo correction of myotonic dystrophy. Nature (2012) 2.63
Plexin-A1 and its interaction with DAP12 in immune responses and bone homeostasis. Nat Cell Biol (2006) 2.57
Signalling mediated by the endoplasmic reticulum stress transducer OASIS is involved in bone formation. Nat Cell Biol (2009) 2.38
OASIS, a CREB/ATF-family member, modulates UPR signalling in astrocytes. Nat Cell Biol (2005) 2.29
Pentamidine reverses the splicing defects associated with myotonic dystrophy. Proc Natl Acad Sci U S A (2009) 2.21
Neuromyelitis optica: Passive transfer to rats by human immunoglobulin. Biochem Biophys Res Commun (2009) 2.20
Intrathecal activation of the IL-17/IL-8 axis in opticospinal multiple sclerosis. Brain (2005) 2.07
Altered mRNA splicing of the skeletal muscle ryanodine receptor and sarcoplasmic/endoplasmic reticulum Ca2+-ATPase in myotonic dystrophy type 1. Hum Mol Genet (2005) 2.07
Class IV semaphorin Sema4A enhances T-cell activation and interacts with Tim-2. Nature (2002) 2.04
SIK2 is a key regulator for neuronal survival after ischemia via TORC1-CREB. Neuron (2011) 2.03
The signalling from endoplasmic reticulum-resident bZIP transcription factors involved in diverse cellular physiology. J Biochem (2011) 1.99
Heterogeneity of aquaporin-4 autoimmunity and spinal cord lesions in multiple sclerosis in Japanese. Brain (2007) 1.93
Misregulated alternative splicing of BIN1 is associated with T tubule alterations and muscle weakness in myotonic dystrophy. Nat Med (2011) 1.87
Muscleblind-like 2-mediated alternative splicing in the developing brain and dysregulation in myotonic dystrophy. Neuron (2012) 1.78
Nationwide survey of juvenile muscular atrophy of distal upper extremity (Hirayama disease) in Japan. Amyotroph Lateral Scler (2006) 1.73
Association of anti-Helicobacter pylori neutrophil-activating protein antibody response with anti-aquaporin-4 autoimmunity in Japanese patients with multiple sclerosis and neuromyelitis optica. Mult Scler (2009) 1.73
Misregulation of miR-1 processing is associated with heart defects in myotonic dystrophy. Nat Struct Mol Biol (2011) 1.73
Endoplasmic reticulum stress response mediated by the PERK-eIF2(alpha)-ATF4 pathway is involved in osteoblast differentiation induced by BMP2. J Biol Chem (2010) 1.72
Regulation of endoplasmic reticulum stress response by a BBF2H7-mediated Sec23a pathway is essential for chondrogenesis. Nat Cell Biol (2009) 1.69
Neuroaxonal dystrophy caused by group VIA phospholipase A2 deficiency in mice: a model of human neurodegenerative disease. J Neurosci (2008) 1.64
Identification of long-term repopulating potential of human cord blood-derived CD34-flt3- severe combined immunodeficiency-repopulating cells by intra-bone marrow injection. Stem Cells (2007) 1.57
Cleavage of the membrane-bound transcription factor OASIS in response to endoplasmic reticulum stress. J Neurochem (2006) 1.56
Different chemokine expression in lethal and non-lethal murine West Nile virus infection. J Med Virol (2004) 1.55
BBF2H7, a novel transmembrane bZIP transcription factor, is a new type of endoplasmic reticulum stress transducer. Mol Cell Biol (2006) 1.52
Intracellular Abeta42 activates p53 promoter: a pathway to neurodegeneration in Alzheimer's disease. FASEB J (2004) 1.51
HLA-B locus in Japanese patients with anti-epileptics and allopurinol-related Stevens-Johnson syndrome and toxic epidermal necrolysis. Pharmacogenomics (2008) 1.49
Role of the prefrontal cortex in human balance control. Neuroimage (2008) 1.48
Expanded CTG repeat demarcates a boundary for abnormal CpG methylation in myotonic dystrophy patient tissues. Hum Mol Genet (2010) 1.46
Design of a bioactive small molecule that targets the myotonic dystrophy type 1 RNA via an RNA motif-ligand database and chemical similarity searching. J Am Chem Soc (2012) 1.44
Molecular mechanisms responsible for aberrant splicing of SERCA1 in myotonic dystrophy type 1. Hum Mol Genet (2007) 1.43
Apoptosis induced by endoplasmic reticulum stress depends on activation of caspase-3 via caspase-12. Neurosci Lett (2004) 1.43
Frequency of chronic headaches in Japanese patients with multiple sclerosis: with special reference to opticospinal and common forms of multiple sclerosis. Headache (2009) 1.42
Induction of neuronal death by ER stress in Alzheimer's disease. J Chem Neuroanat (2004) 1.42
Obesity-induced endoplasmic reticulum stress causes chronic inflammation in adipose tissue. Sci Rep (2012) 1.42
Extensive distribution of glial cytoplasmic inclusions in an autopsied case of multiple system atrophy with a prolonged 18-year clinical course. Neuropathology (2011) 1.41
Anti-aquaporin-4 antibody induces astrocytic cytotoxicity in the absence of CNS antigen-specific T cells. Biochem Biophys Res Commun (2010) 1.40
Muscle weakness in myotonic dystrophy associated with misregulated splicing and altered gating of Ca(V)1.1 calcium channel. Hum Mol Genet (2011) 1.37
Proteomic analysis of human brain identifies alpha-enolase as a novel autoantigen in Hashimoto's encephalopathy. FEBS Lett (2002) 1.37
Identification of a cis-acting element for the regulation of SMN exon 7 splicing. J Biol Chem (2002) 1.33
Triadin binding to the C-terminal luminal loop of the ryanodine receptor is important for skeletal muscle excitation contraction coupling. J Gen Physiol (2007) 1.30
MDG1/ERdj4, an ER-resident DnaJ family member, suppresses cell death induced by ER stress. Genes Cells (2003) 1.29
An intronic splicing enhancer element in survival motor neuron (SMN) pre-mRNA. J Biol Chem (2003) 1.28
A unique case of fibrodysplasia ossificans progressiva with an ACVR1 mutation, G356D, other than the common mutation (R206H). Am J Med Genet A (2008) 1.27
The human polyoma JC virus agnoprotein acts as a viroporin. PLoS Pathog (2010) 1.26
Dynamics of coupled vortices in a pair of ferromagnetic disks. Phys Rev Lett (2011) 1.25
JAB1 participates in unfolded protein responses by association and dissociation with IRE1. Neurochem Int (2004) 1.21
Benefit of valproic acid in suppressing disease progression of ALS model mice. Eur J Neurosci (2004) 1.21
Astrocytic necrosis is induced by anti-aquaporin-4 antibody-positive serum. Neuroreport (2009) 1.19
Multiple system degeneration with basophilic inclusions in Japanese ALS patients with FUS mutation. Acta Neuropathol (2009) 1.16
HLA-B*1511 is a risk factor for carbamazepine-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in Japanese patients. Epilepsia (2010) 1.16
Characteristics of myasthenia gravis according to onset-age: Japanese nationwide survey. J Neurol Sci (2011) 1.15
Neuroaxonal dystrophy in calcium-independent phospholipase A2β deficiency results from insufficient remodeling and degeneration of mitochondrial and presynaptic membranes. J Neurosci (2011) 1.14
A novel method for systematic analysis of rigidity in Parkinson's disease. Mov Disord (2009) 1.14
Hepatoma-derived growth factor is a neurotrophic factor harbored in the nucleus. J Biol Chem (2004) 1.14
Ssdp1 regulates head morphogenesis of mouse embryos by activating the Lim1-Ldb1 complex. Development (2005) 1.12
A case of hereditary diffuse leukoencephalopathy with axonal spheroids caused by a de novo mutation in CSF1R masquerading as primary progressive multiple sclerosis. Mult Scler (2013) 1.12
Targeted lipidomics reveals mPGES-1-PGE2 as a therapeutic target for multiple sclerosis. Proc Natl Acad Sci U S A (2009) 1.12
Effect of an inducer of BiP, a molecular chaperone, on endoplasmic reticulum (ER) stress-induced retinal cell death. Invest Ophthalmol Vis Sci (2008) 1.11
Large T antigen promotes JC virus replication in G2-arrested cells by inducing ATM- and ATR-mediated G2 checkpoint signaling. J Biol Chem (2009) 1.11
Tra2 beta, SF2/ASF and SRp30c modulate the function of an exonic splicing enhancer in exon 10 of tau pre-mRNA. Genes Cells (2004) 1.11
XBP1 activates the transcription of its target genes via an ACGT core sequence under ER stress. Biochem Biophys Res Commun (2005) 1.10
From dynamic combinatorial 'hit' to lead: in vitro and in vivo activity of compounds targeting the pathogenic RNAs that cause myotonic dystrophy. Nucleic Acids Res (2012) 1.10
Reappraisal of the motor role of basal ganglia: a functional magnetic resonance image study. J Neurosci (2003) 1.08
Proton MR spectroscopic study at 3 Tesla on glutamate/glutamine in Alzheimer's disease. Neuroreport (2002) 1.08
TDP-43 pathology in sporadic ALS occurs in motor neurons lacking the RNA editing enzyme ADAR2. Acta Neuropathol (2010) 1.06
Multiple therapeutic peptide vaccines consisting of combined novel cancer testis antigens and anti-angiogenic peptides for patients with non-small cell lung cancer. J Transl Med (2013) 1.06
The expanding phenotype of CLIPPERS: is it a disease or a syndrome? J Neurol Neurosurg Psychiatry (2012) 1.06
Fibroblast growth factor 20 gene and Parkinson's disease in the Japanese population. Neuroreport (2007) 1.05
A unique mutation of ALK2, G356D, found in a patient with fibrodysplasia ossificans progressiva is a moderately activated BMP type I receptor. Biochem Biophys Res Commun (2008) 1.05
Muscleblind-like 1 knockout mice reveal novel splicing defects in the myotonic dystrophy brain. PLoS One (2012) 1.05
Mice lacking the schizophrenia-associated protein FEZ1 manifest hyperactivity and enhanced responsiveness to psychostimulants. Hum Mol Genet (2008) 1.05
Physiological unfolded protein response regulated by OASIS family members, transmembrane bZIP transcription factors. IUBMB Life (2011) 1.05