Published in Nat Med on May 18, 2003
Genetics of motor neuron disorders: new insights into pathogenic mechanisms. Nat Rev Genet (2009) 2.31
Overexpression of IGF-1 in muscle attenuates disease in a mouse model of spinal and bulbar muscular atrophy. Neuron (2009) 2.26
Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions. Proc Natl Acad Sci U S A (2005) 1.87
Clinical features of spinal and bulbar muscular atrophy. Brain (2009) 1.75
Androgen-dependent pathology demonstrates myopathic contribution to the Kennedy disease phenotype in a mouse knock-in model. J Clin Invest (2006) 1.67
Mitochondrial abnormalities in spinal and bulbar muscular atrophy. Hum Mol Genet (2008) 1.56
TDP-43 depletion induces neuronal cell damage through dysregulation of Rho family GTPases. J Biol Chem (2009) 1.44
Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease. Proc Natl Acad Sci U S A (2005) 1.42
Viral delivery of miR-196a ameliorates the SBMA phenotype via the silencing of CELF2. Nat Med (2012) 1.41
Cytoplasmic retention of polyglutamine-expanded androgen receptor ameliorates disease via autophagy in a mouse model of spinal and bulbar muscular atrophy. Hum Mol Genet (2009) 1.32
Post-translational modifications of nuclear receptors and human disease. Nucl Recept Signal (2012) 1.23
SIRT1 in neurodevelopment and brain senescence. Neuron (2014) 1.22
Small ubiquitin-like modifier (SUMO) modification of the androgen receptor attenuates polyglutamine-mediated aggregation. J Biol Chem (2009) 1.19
Sirtuin deacetylases in neurodegenerative diseases of aging. Cell Res (2013) 1.09
An interdomain interaction of the androgen receptor is required for its aggregation and toxicity in spinal and bulbar muscular atrophy. J Biol Chem (2010) 1.03
Spinal and bulbar muscular atrophy: a motoneuron or muscle disease? Curr Opin Pharmacol (2008) 1.01
Enhanced aggregation of androgen receptor in induced pluripotent stem cell-derived neurons from spinal and bulbar muscular atrophy. J Biol Chem (2013) 0.99
Mutant huntingtin impairs Ku70-mediated DNA repair. J Cell Biol (2010) 0.97
Insulinlike growth factor (IGF)-1 administration ameliorates disease manifestations in a mouse model of spinal and bulbar muscular atrophy. Mol Med (2012) 0.96
Transcriptional repression induces a slowly progressive atypical neuronal death associated with changes of YAP isoforms and p73. J Cell Biol (2006) 0.95
dnc-1/dynactin 1 knockdown disrupts transport of autophagosomes and induces motor neuron degeneration. PLoS One (2013) 0.94
Developing treatment for spinal and bulbar muscular atrophy. Prog Neurobiol (2012) 0.92
Getting a handle on Huntington's disease: the case for cholesterol. Nat Med (2009) 0.92
Naratriptan mitigates CGRP1-associated motor neuron degeneration caused by an expanded polyglutamine repeat tract. Nat Med (2012) 0.91
Protein arginine methyltransferase 6 enhances polyglutamine-expanded androgen receptor function and toxicity in spinal and bulbar muscular atrophy. Neuron (2015) 0.90
Skeletal muscle in motor neuron diseases: therapeutic target and delivery route for potential treatments. Curr Drug Targets (2010) 0.89
Spinal and bulbar muscular atrophy: pathogenesis and clinical management. Oral Dis (2013) 0.89
Heat shock factor-1 influences pathological lesion distribution of polyglutamine-induced neurodegeneration. Nat Commun (2013) 0.88
Sex steroid-related candidate genes in psychiatric disorders. J Psychiatry Neurosci (2008) 0.88
Prenatal flutamide enhances survival in a myogenic mouse model of spinal bulbar muscular atrophy. Neurodegener Dis (2010) 0.87
Mouse models of polyglutamine diseases in therapeutic approaches: review and data table. Part II. Mol Neurobiol (2012) 0.86
Clearance of the mutant androgen receptor in motoneuronal models of spinal and bulbar muscular atrophy. Neurobiol Aging (2013) 0.85
B2 attenuates polyglutamine-expanded androgen receptor toxicity in cell and fly models of spinal and bulbar muscular atrophy. J Neurosci Res (2010) 0.85
Drosophila as an In Vivo Model for Human Neurodegenerative Disease. Genetics (2015) 0.83
Antiandrogen flutamide protects male mice from androgen-dependent toxicity in three models of spinal bulbar muscular atrophy. Endocrinology (2014) 0.81
Pathogenic mechanisms and therapeutic strategies in spinobulbar muscular atrophy. CNS Neurol Disord Drug Targets (2013) 0.80
Human adipose-derived mesenchymal stem cells as a new model of spinal and bulbar muscular atrophy. PLoS One (2014) 0.80
The hnRNP-Htt axis regulates necrotic cell death induced by transcriptional repression through impaired RNA splicing. Cell Death Dis (2016) 0.80
Current status of treatment of spinal and bulbar muscular atrophy. Neural Plast (2012) 0.78
Testosterone treatment fails to accelerate disease in a transgenic mouse model of spinal and bulbar muscular atrophy. Dis Model Mech (2011) 0.78
Nucleocytoplasmic transfer of cyclin dependent kinase 5 and its binding to puromycin-sensitive aminopeptidase in Dictyostelium discoideum. Histochem Cell Biol (2011) 0.78
Neuropathology and therapeutic intervention in spinal and bulbar muscular atrophy. Int J Mol Sci (2009) 0.78
Increased mitophagy in the skeletal muscle of spinal and bulbar muscular atrophy patients. Hum Mol Genet (2017) 0.77
Difference in chronological changes of outcome measures between untreated and placebo-treated patients of spinal and bulbar muscular atrophy. J Neurol (2011) 0.77
Animal models of Kennedy disease. NeuroRx (2005) 0.77
Widespread white matter changes in Kennedy disease: a voxel based morphometry study. J Neurol Neurosurg Psychiatry (2007) 0.75
Spinal and Bulbar Muscular Atrophy. Neurol Clin (2015) 0.75
New prospects and strategies for drug target discovery in neurodegenerative disorders. NeuroRx (2005) 0.75
Anti-androgen treatment for spinal and bulbar muscular atrophy. Ann Neurol (2009) 0.75
Spinal and Bulbar Muscular Atrophy Overview. J Mol Neurosci (2015) 0.75
Convenient diagnosis of spinal and bulbar muscular atrophy using a microchip electrophoresis system. Am J Neurodegener Dis (2013) 0.75
Trinucleotide repeats and protein folding and disease: the perspective from studies with the androgen receptor. Future Sci OA (2015) 0.75
Swallowing markers in spinal and bulbar muscular atrophy. Ann Clin Transl Neurol (2017) 0.75
(99m)Tc-LHRH in tumor receptor imaging. Oncol Lett (2017) 0.75
Identification of neuron selective androgen receptor inhibitors. World J Biol Chem (2017) 0.75
The Role of the Heat Shock Protein B8 (HSPB8) in Motoneuron Diseases. Front Mol Neurosci (2017) 0.75
FD, a bZIP protein mediating signals from the floral pathway integrator FT at the shoot apex. Science (2005) 6.38
Hd3a, a rice ortholog of the Arabidopsis FT gene, promotes transition to flowering downstream of Hd1 under short-day conditions. Plant Cell Physiol (2002) 4.49
The wide spectrum of clinical manifestations in Sjögren's syndrome-associated neuropathy. Brain (2005) 3.65
Th17 cells express interleukin-10 receptor and are controlled by Foxp3⁻ and Foxp3+ regulatory CD4+ T cells in an interleukin-10-dependent manner. Immunity (2011) 3.38
17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration. Nat Med (2005) 3.19
Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Neuron (2002) 2.98
TWIN SISTER OF FT (TSF) acts as a floral pathway integrator redundantly with FT. Plant Cell Physiol (2005) 2.80
Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients. Brain (2002) 2.35
Efficacy and safety of leuprorelin in patients with spinal and bulbar muscular atrophy (JASMITT study): a multicentre, randomised, double-blind, placebo-controlled trial. Lancet Neurol (2010) 2.33
Overexpression of IGF-1 in muscle attenuates disease in a mouse model of spinal and bulbar muscular atrophy. Neuron (2009) 2.26
Retinoic-acid-concentration-dependent acquisition of neural cell identity during in vitro differentiation of mouse embryonic stem cells. Dev Biol (2004) 2.17
ASC-J9 ameliorates spinal and bulbar muscular atrophy phenotype via degradation of androgen receptor. Nat Med (2007) 1.87
Natural history of transthyretin Val30Met familial amyloid polyneuropathy: analysis of late-onset cases from non-endemic areas. J Neurol Neurosurg Psychiatry (2012) 1.81
Nationwide survey of juvenile muscular atrophy of distal upper extremity (Hirayama disease) in Japan. Amyotroph Lateral Scler (2006) 1.73
Usefulness of combined fractional anisotropy and apparent diffusion coefficient values for detection of involvement in multiple system atrophy. J Neurol Neurosurg Psychiatry (2007) 1.69
Spatiotemporal recapitulation of central nervous system development by murine embryonic stem cell-derived neural stem/progenitor cells. Stem Cells (2008) 1.67
Differential expression of inflammation- and apoptosis-related genes in spinal cords of a mutant SOD1 transgenic mouse model of familial amyotrophic lateral sclerosis. J Neurochem (2002) 1.63
Gene expression profile of spinal motor neurons in sporadic amyotrophic lateral sclerosis. Ann Neurol (2005) 1.59
Position-dependent FUS-RNA interactions regulate alternative splicing events and transcriptions. Sci Rep (2012) 1.58
Clinical and neuropathological correlates of Lewy body disease. Acta Neuropathol (2003) 1.58
Natural history of spinal and bulbar muscular atrophy (SBMA): a study of 223 Japanese patients. Brain (2006) 1.57
Disulfide bond mediates aggregation, toxicity, and ubiquitylation of familial amyotrophic lateral sclerosis-linked mutant SOD1. J Biol Chem (2007) 1.57
Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein. J Neurosci (2003) 1.54
Sodium butyrate ameliorates phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Hum Mol Genet (2004) 1.52
Suppression of UV-induced wrinkle formation by induction of HSP70 expression in mice. J Invest Dermatol (2012) 1.46
TDP-43 depletion induces neuronal cell damage through dysregulation of Rho family GTPases. J Biol Chem (2009) 1.44
Phase 2 trial of leuprorelin in patients with spinal and bulbar muscular atrophy. Ann Neurol (2009) 1.43
Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease. Proc Natl Acad Sci U S A (2005) 1.42
Extracellular signal-regulated kinase 2 (ERK2) knockdown mice show deficits in long-term memory; ERK2 has a specific function in learning and memory. J Neurosci (2007) 1.41
Viral delivery of miR-196a ameliorates the SBMA phenotype via the silencing of CELF2. Nat Med (2012) 1.41
Alcoholic neuropathy is clinicopathologically distinct from thiamine-deficiency neuropathy. Ann Neurol (2003) 1.39
Early pathologic changes in hereditary diffuse leukoencephalopathy with spheroids. J Neuropathol Exp Neurol (2014) 1.38
Different pedunculopontine tegmental neurons signal predicted and actual task rewards. J Neurosci (2009) 1.36
Genetic evidence for a protective role for heat shock factor 1 and heat shock protein 70 against colitis. J Biol Chem (2007) 1.36
Structure of HIV-1 protease in complex with potent inhibitor KNI-272 determined by high-resolution X-ray and neutron crystallography. Proc Natl Acad Sci U S A (2009) 1.34
CHIP overexpression reduces mutant androgen receptor protein and ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model. J Neurosci (2007) 1.34
Widespread nuclear and cytoplasmic accumulation of mutant androgen receptor in SBMA patients. Brain (2005) 1.33
Cis-regulatory changes at FLOWERING LOCUS T mediate natural variation in flowering responses of Arabidopsis thaliana. Genetics (2009) 1.28
Paraneoplastic neuropathy: wide-ranging clinicopathological manifestations. Curr Opin Neurol (2011) 1.26
17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in an SBMA model mouse. Hum Mol Genet (2008) 1.25
Reversible disruption of dynactin 1-mediated retrograde axonal transport in polyglutamine-induced motor neuron degeneration. J Neurosci (2006) 1.25
Familial transthyretin-type amyloid polyneuropathy in Japan: clinical and genetic heterogeneity. Neurology (2002) 1.24
Disease progression of human SOD1 (G93A) transgenic ALS model rats. J Neurosci Res (2006) 1.24
Ectopic expression of an FT homolog from citrus confers an early flowering phenotype on trifoliate orange (Poncirus trifoliata L. Raf.). Transgenic Res (2005) 1.23
Physical and functional interaction between Dorfin and Valosin-containing protein that are colocalized in ubiquitylated inclusions in neurodegenerative disorders. J Biol Chem (2004) 1.23
Spliceosome integrity is defective in the motor neuron diseases ALS and SMA. EMBO Mol Med (2013) 1.22
Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity. J Biol Chem (2002) 1.19
Type I (transthyretin Met30) familial amyloid polyneuropathy in Japan: early- vs late-onset form. Arch Neurol (2002) 1.19
Pathogenesis, animal models and therapeutics in spinal and bulbar muscular atrophy (SBMA). Exp Neurol (2006) 1.18