Published in J Biol Chem on December 02, 2002
Atrophin proteins: an overview of a new class of nuclear receptor corepressors. Nucl Recept Signal (2008) 1.10
Proteotoxic stress increases nuclear localization of ataxin-3. Hum Mol Genet (2009) 1.02
DFNA5: hearing impairment exon instead of hearing impairment gene? J Med Genet (2004) 0.93
Non-targeted identification of prions and amyloid-forming proteins from yeast and mammalian cells. J Biol Chem (2013) 0.89
Severe neurological phenotypes of Q129 DRPLA transgenic mice serendipitously created by en masse expansion of CAG repeats in Q76 DRPLA mice. Hum Mol Genet (2008) 0.86
Nuclear accumulation of polyglutamine disease proteins and neuropathology. Mol Brain (2009) 0.85
Polyglutamine expansion alters the dynamics and molecular architecture of aggregates in dentatorubropallidoluysian atrophy. J Biol Chem (2011) 0.81
Pathological accumulation of atrophin-1 in dentatorubralpallidoluysian atrophy. Int J Clin Exp Pathol (2011) 0.80
Amyloid cannot resist identification. Prion (2013) 0.78
Ataxin-2 mediated cell death is dependent on domains downstream of the polyQ repeat. Exp Neurol (2007) 0.78
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A Drosophila model of Huntington disease-like 2 exhibits nuclear toxicity and distinct pathogenic mechanisms from Huntington disease. Hum Mol Genet (2016) 0.75
Guidelines for the use and interpretation of assays for monitoring autophagy. Autophagy (2012) 20.08
Mediation of poly(ADP-ribose) polymerase-1-dependent cell death by apoptosis-inducing factor. Science (2002) 8.41
PINK1-dependent recruitment of Parkin to mitochondria in mitophagy. Proc Natl Acad Sci U S A (2009) 8.25
Molecular pathways of neurodegeneration in Parkinson's disease. Science (2003) 7.55
S-nitrosylated GAPDH initiates apoptotic cell death by nuclear translocation following Siah1 binding. Nat Cell Biol (2005) 7.25
Parkinson's disease-associated mutations in leucine-rich repeat kinase 2 augment kinase activity. Proc Natl Acad Sci U S A (2005) 6.98
Molecular pathophysiology of Parkinson's disease. Annu Rev Neurosci (2005) 5.93
Mutations in HFE2 cause iron overload in chromosome 1q-linked juvenile hemochromatosis. Nat Genet (2003) 5.82
Kinase activity of mutant LRRK2 mediates neuronal toxicity. Nat Neurosci (2006) 5.32
Parkinson's disease-associated mutations in LRRK2 link enhanced GTP-binding and kinase activities to neuronal toxicity. Hum Mol Genet (2007) 4.99
S-nitrosylation of parkin regulates ubiquitination and compromises parkin's protective function. Science (2004) 4.97
Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes. Nat Genet (2003) 4.76
Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell (2006) 4.53
Inhibitors of leucine-rich repeat kinase-2 protect against models of Parkinson's disease. Nat Med (2010) 4.45
Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nat Neurosci (2002) 4.15
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Hum Mol Genet (2003) 4.07
PARIS (ZNF746) repression of PGC-1α contributes to neurodegeneration in Parkinson's disease. Cell (2011) 4.00
Parkin mediates nonclassical, proteasomal-independent ubiquitination of synphilin-1: implications for Lewy body formation. J Neurosci (2005) 3.92
A schizophrenia-associated mutation of DISC1 perturbs cerebral cortex development. Nat Cell Biol (2005) 3.89
Genetic animal models of Parkinson's disease. Neuron (2010) 3.86
Genetic variants in TPMT and COMT are associated with hearing loss in children receiving cisplatin chemotherapy. Nat Genet (2009) 3.83
Localization of LRRK2 to membranous and vesicular structures in mammalian brain. Ann Neurol (2006) 3.72
Preparing for preventive clinical trials: the Predict-HD study. Arch Neurol (2006) 3.57
Beta-cell ABCA1 influences insulin secretion, glucose homeostasis and response to thiazolidinedione treatment. Nat Med (2007) 3.53
Apoptosis-inducing factor mediates poly(ADP-ribose) (PAR) polymer-induced cell death. Proc Natl Acad Sci U S A (2006) 3.47
Poly(ADP-ribose) (PAR) polymer is a death signal. Proc Natl Acad Sci U S A (2006) 3.41
Disrupted-in-Schizophrenia-1 (DISC-1): mutant truncation prevents binding to NudE-like (NUDEL) and inhibits neurite outgrowth. Proc Natl Acad Sci U S A (2002) 3.34
Human alpha-synuclein-harboring familial Parkinson's disease-linked Ala-53 --> Thr mutation causes neurodegenerative disease with alpha-synuclein aggregation in transgenic mice. Proc Natl Acad Sci U S A (2002) 3.33
Targeted inactivation of hepatic Abca1 causes profound hypoalphalipoproteinemia and kidney hypercatabolism of apoA-I. J Clin Invest (2005) 3.25
Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1. Neuron (2003) 3.25
Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease. Neuron (2002) 3.15
p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease. Neuron (2005) 3.13
CHIP and Hsp70 regulate tau ubiquitination, degradation and aggregation. Hum Mol Genet (2004) 3.12
Huntingtin-interacting protein HIP14 is a palmitoyl transferase involved in palmitoylation and trafficking of multiple neuronal proteins. Neuron (2004) 3.04
Dominant-negative DISC1 transgenic mice display schizophrenia-associated phenotypes detected by measures translatable to humans. Proc Natl Acad Sci U S A (2007) 3.02
Leucine-rich repeat kinase 2 (LRRK2) interacts with parkin, and mutant LRRK2 induces neuronal degeneration. Proc Natl Acad Sci U S A (2005) 3.00
Differential modulation of endotoxin responsiveness by human caspase-12 polymorphisms. Nature (2004) 2.98
Mutant frizzled-4 disrupts retinal angiogenesis in familial exudative vitreoretinopathy. Nat Genet (2002) 2.90
Disrupted-in-Schizophrenia 1 (DISC1) regulates spines of the glutamate synapse via Rac1. Nat Neurosci (2010) 2.90
Linking neurodevelopmental and synaptic theories of mental illness through DISC1. Nat Rev Neurosci (2011) 2.89
DJ-1 gene deletion reveals that DJ-1 is an atypical peroxiredoxin-like peroxidase. Proc Natl Acad Sci U S A (2007) 2.87
Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity. Nat Med (2011) 2.87
Diagnosis and treatment of Parkinson disease: molecules to medicine. J Clin Invest (2006) 2.86
Central nervous system depression of neonates breastfed by mothers receiving oxycodone for postpartum analgesia. J Pediatr (2011) 2.86
A hierarchical NGF signaling cascade controls Ret-dependent and Ret-independent events during development of nonpeptidergic DRG neurons. Neuron (2007) 2.83
A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J Exp Med (2008) 2.78
Huntingtin interacting proteins are genetic modifiers of neurodegeneration. PLoS Genet (2007) 2.77
Mitochondrial localization of the Parkinson's disease related protein DJ-1: implications for pathogenesis. Hum Mol Genet (2005) 2.72
Coupling endoplasmic reticulum stress to the cell death program: role of the ER chaperone GRP78. FEBS Lett (2002) 2.71
Proteome-wide identification of poly(ADP-ribose) binding proteins and poly(ADP-ribose)-associated protein complexes. Nucleic Acids Res (2008) 2.70
Increased susceptibility of cytoplasmic over nuclear polyglutamine aggregates to autophagic degradation. Proc Natl Acad Sci U S A (2005) 2.69
Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin. Nat Med (2009) 2.66
Nitric oxide-induced nuclear GAPDH activates p300/CBP and mediates apoptosis. Nat Cell Biol (2008) 2.56
Lipoprotein lipase S447X: a naturally occurring gain-of-function mutation. Arterioscler Thromb Vasc Biol (2006) 2.55
Neurodevelopmental mechanisms of schizophrenia: understanding disturbed postnatal brain maturation through neuregulin-1-ErbB4 and DISC1. Trends Neurosci (2009) 2.55
Intestinal ABCA1 directly contributes to HDL biogenesis in vivo. J Clin Invest (2006) 2.55
Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage. Hum Mol Genet (2007) 2.54
Knockdown of DISC1 by in utero gene transfer disturbs postnatal dopaminergic maturation in the frontal cortex and leads to adult behavioral deficits. Neuron (2010) 2.53
Role of AIF in caspase-dependent and caspase-independent cell death. Oncogene (2004) 2.52
Psychopathology in patients with degenerative cerebellar diseases: a comparison to Huntington's disease. Am J Psychiatry (2002) 2.52
Androgen receptor YAC transgenic mice recapitulate SBMA motor neuronopathy and implicate VEGF164 in the motor neuron degeneration. Neuron (2004) 2.52
Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi. Nat Cell Biol (2002) 2.50
KCTD13 is a major driver of mirrored neuroanatomical phenotypes of the 16p11.2 copy number variant. Nature (2012) 2.47
Parkin protects against LRRK2 G2019S mutant-induced dopaminergic neurodegeneration in Drosophila. J Neurosci (2009) 2.47
Apoptosis-inducing factor is involved in the regulation of caspase-independent neuronal cell death. J Cell Biol (2002) 2.47
Aggregation promoting C-terminal truncation of alpha-synuclein is a normal cellular process and is enhanced by the familial Parkinson's disease-linked mutations. Proc Natl Acad Sci U S A (2005) 2.45
Lysine 63-linked ubiquitination promotes the formation and autophagic clearance of protein inclusions associated with neurodegenerative diseases. Hum Mol Genet (2007) 2.44