Published in Hum Mol Genet on April 01, 2008
Toward understanding Machado-Joseph disease. Prog Neurobiol (2011) 1.42
Allele-specific RNA silencing of mutant ataxin-3 mediates neuroprotection in a rat model of Machado-Joseph disease. PLoS One (2008) 1.34
Conformational targeting of fibrillar polyglutamine proteins in live cells escalates aggregation and cytotoxicity. PLoS One (2009) 1.01
Neuron-specific proteotoxicity of mutant ataxin-3 in C. elegans: rescue by the DAF-16 and HSF-1 pathways. Hum Mol Genet (2011) 1.00
Silencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic mice. PLoS One (2013) 0.96
Distinct transduction profiles in the CNS via three injection routes of AAV9 and the application to generation of a neurodegenerative mouse model. Mol Ther Methods Clin Dev (2014) 0.93
Ubiquitin-binding site 2 of ataxin-3 prevents its proteasomal degradation by interacting with Rad23. Nat Commun (2014) 0.90
Mouse models of polyglutamine diseases: review and data table. Part I. Mol Neurobiol (2012) 0.84
Mouse ataxin-3 functional knock-out model. Neuromolecular Med (2010) 0.84
Mouse models of spinocerebellar ataxia type 3 (Machado-Joseph disease). Neurotherapeutics (2012) 0.80
Nucleocytoplasmic shuttling activity of ataxin-3. PLoS One (2009) 0.79
Ataxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia type 3 models. J Cell Biol (2016) 0.77
Lentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteins. Mol Neurodegener (2016) 0.77
Caloric restriction blocks neuropathology and motor deficits in Machado-Joseph disease mouse models through SIRT1 pathway. Nat Commun (2016) 0.76
RNA interference mitigates motor and neuropathological deficits in a cerebellar mouse model of Machado-Joseph disease. PLoS One (2014) 0.76
Adenosine A2A Receptors in the Amygdala Control Synaptic Plasticity and Contextual Fear Memory. Neuropsychopharmacology (2016) 0.75
Unravelling Endogenous MicroRNA System Dysfunction as a New Pathophysiological Mechanism in Machado-Joseph Disease. Mol Ther (2017) 0.75
Paclitaxel-loaded PLGA nanoparticles: preparation, physicochemical characterization and in vitro anti-tumoral activity. J Control Release (2002) 2.21
Sustained effects of nonallele-specific Huntingtin silencing. Ann Neurol (2009) 2.05
Lentiviral-mediated RNA interference. Hum Gene Ther (2002) 1.99
On the formulation of pH-sensitive liposomes with long circulation times. Adv Drug Deliv Rev (2004) 1.71
Single-step detection of mutant huntingtin in animal and human tissues: a bioassay for Huntington's disease. Anal Biochem (2009) 1.64
Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease. J Neurochem (2007) 1.54
Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin. Mol Biol Cell (2006) 1.51
Brain-derived neurotrophic factor regulates the expression and synaptic delivery of alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid receptor subunits in hippocampal neurons. J Biol Chem (2007) 1.46
Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase. J Clin Invest (2006) 1.44
Mitochondria in Huntington's disease. Biochim Biophys Acta (2009) 1.41
Lentiviral-mediated delivery of mutant huntingtin in the striatum of rats induces a selective neuropathology modulated by polyglutamine repeat size, huntingtin expression levels, and protein length. J Neurosci (2002) 1.40
Hsp104 antagonizes alpha-synuclein aggregation and reduces dopaminergic degeneration in a rat model of Parkinson disease. J Clin Invest (2008) 1.38
Progressive and selective striatal degeneration in primary neuronal cultures using lentiviral vector coding for a mutant huntingtin fragment. Neurobiol Dis (2005) 1.37
Analysis of potential transcriptomic biomarkers for Huntington's disease in peripheral blood. Proc Natl Acad Sci U S A (2007) 1.35
In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington's disease subjects. Hum Mol Genet (2010) 1.34
Allele-specific RNA silencing of mutant ataxin-3 mediates neuroprotection in a rat model of Machado-Joseph disease. PLoS One (2008) 1.34
Akt is altered in an animal model of Huntington's disease and in patients. Eur J Neurosci (2005) 1.27
Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's disease. Mol Ther (2007) 1.26
Dopamine gene therapy for Parkinson's disease in a nonhuman primate without associated dyskinesia. Sci Transl Med (2009) 1.25
Suicide gene therapy in cancer: where do we stand now? Cancer Lett (2012) 1.25
Engineered lentiviral vector targeting astrocytes in vivo. Glia (2009) 1.24
Human and simian immunodeficiency viruses deregulate early hematopoiesis through a Nef/PPARgamma/STAT5 signaling pathway in macaques. J Clin Invest (2008) 1.24
BDNF regulates the expression and traffic of NMDA receptors in cultured hippocampal neurons. Mol Cell Neurosci (2007) 1.24
Dysregulation of gene expression in primary neuron models of Huntington's disease shows that polyglutamine-related effects on the striatal transcriptome may not be dependent on brain circuitry. J Neurosci (2008) 1.23
Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado-Joseph disease. Brain (2011) 1.17
Inhibition of calcineurin by FK506 protects against polyglutamine-huntingtin toxicity through an increase of huntingtin phosphorylation at S421. J Neurosci (2006) 1.16
Sterols as anticancer agents: synthesis of ring-B oxygenated steroids, cytotoxic profile, and comprehensive SAR analysis. J Med Chem (2010) 1.14
Decreased TCA cycle rate in the rat brain after acute 3-NP treatment measured by in vivo 1H-[13C] NMR spectroscopy. J Neurochem (2002) 1.14
A role of mitochondrial complex II defects in genetic models of Huntington's disease expressing N-terminal fragments of mutant huntingtin. Hum Mol Genet (2013) 1.13
Applications of lentiviral vectors for biology and gene therapy of neurological disorders. Curr Gene Ther (2008) 1.12
Calpain is a major cell death effector in selective striatal degeneration induced in vivo by 3-nitropropionate: implications for Huntington's disease. J Neurosci (2003) 1.12
Cannabinoid CB2 receptor agonists protect the striatum against malonate toxicity: relevance for Huntington's disease. Glia (2009) 1.11
Activation of astrocytes by CNTF induces metabolic plasticity and increases resistance to metabolic insults. J Neurosci (2007) 1.11
Lentivirally delivered glial cell line-derived neurotrophic factor increases the number of striatal dopaminergic neurons in primate models of nigrostriatal degeneration. J Neurosci (2002) 1.10
Early and reversible neuropathology induced by tetracycline-regulated lentiviral overexpression of mutant huntingtin in rat striatum. Hum Mol Genet (2003) 1.10
Multiply attenuated, self-inactivating lentiviral vectors efficiently deliver and express genes for extended periods of time in adult rat cardiomyocytes in vivo. Circulation (2003) 1.09
Neuron-to-neuron wild-type Tau protein transfer through a trans-synaptic mechanism: relevance to sporadic tauopathies. Acta Neuropathol Commun (2014) 1.09
Lentivirus-mediated expression of glutathione peroxidase: neuroprotection in murine models of Parkinson's disease. Neurobiol Dis (2005) 1.07
Human alpha-iduronidase gene transfer mediated by adeno-associated virus types 1, 2, and 5 in the brain of nonhuman primates: vector diffusion and biodistribution. Hum Gene Ther (2009) 1.06
Silencing ataxin-3 mitigates degeneration in a rat model of Machado-Joseph disease: no role for wild-type ataxin-3? Hum Mol Genet (2010) 1.06
A novel systemically active caspase inhibitor attenuates the toxicities of MPTP, malonate, and 3NP in vivo. Neurobiol Dis (2004) 1.06
The dominant form of vanishing white matter-like leukoencephalopathy represents autosomal dominant leukodystrophy. Ann Neurol (2006) 1.06
Early alterations of brain cellular energy homeostasis in Huntington disease models. J Biol Chem (2011) 1.05
Ciliary neurotrophic factor activates astrocytes, redistributes their glutamate transporters GLAST and GLT-1 to raft microdomains, and improves glutamate handling in vivo. J Neurosci (2006) 1.04
Bcl-2-targeted antisense therapy (Oblimersen sodium): towards clinical reality. Rev Recent Clin Trials (2006) 1.01
Neurospheres modified to produce glial cell line-derived neurotrophic factor increase the survival of transplanted dopamine neurons. J Neurosci Res (2002) 1.01
Delivery of ciliary neurotrophic factor via lentiviral-mediated transfer protects axotomized retinal ganglion cells for an extended period of time. Hum Gene Ther (2003) 0.99
Seizure suppression by adenosine-releasing cells is independent of seizure frequency. Epilepsia (2002) 0.99
A dual role of adenosine A2A receptors in 3-nitropropionic acid-induced striatal lesions: implications for the neuroprotective potential of A2A antagonists. J Neurosci (2003) 0.98
Minocycline in phenotypic models of Huntington's disease. Neurobiol Dis (2005) 0.98
Protein kinase C gamma associates directly with the GluR4 alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionate receptor subunit. Effect on receptor phosphorylation. J Biol Chem (2002) 0.98
NEDD8: a new ataxin-3 interactor. Biochim Biophys Acta (2007) 0.98
Polyglutamine diseases: the special case of ataxin-3 and Machado-Joseph disease. Prog Neurobiol (2011) 0.98
Lipid-based nanoparticles for siRNA delivery in cancer therapy: paradigms and challenges. Acc Chem Res (2012) 0.97
Silencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic mice. PLoS One (2013) 0.96
In vivo calpain/caspase cross-talk during 3-nitropropionic acid-induced striatal degeneration: implication of a calpain-mediated cleavage of active caspase-3. J Biol Chem (2003) 0.96
The mitochondrial toxin 3-nitropropionic acid induces striatal neurodegeneration via a c-Jun N-terminal kinase/c-Jun module. J Neurosci (2002) 0.96
The first crystal structure of class III superoxide reductase from Treponema pallidum. J Biol Inorg Chem (2006) 0.95
Nuclear factor erythroid 2-related factor 2 facilitates neuronal glutathione synthesis by upregulating neuronal excitatory amino acid transporter 3 expression. J Neurosci (2011) 0.95
Diminished hippocalcin expression in Huntington's disease brain does not account for increased striatal neuron vulnerability as assessed in primary neurons. J Neurochem (2009) 0.95
CA150 expression delays striatal cell death in overexpression and knock-in conditions for mutant huntingtin neurotoxicity. J Neurosci (2006) 0.95
Expression of mutated huntingtin fragment in the putamen is sufficient to produce abnormal movement in non-human primates. Mol Ther (2007) 0.94