Glutamine repeats and neurodegeneration.

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Native functions of the androgen receptor are essential to pathogenesis in a Drosophila model of spinobulbar muscular atrophy. Neuron (2010) 1.75

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Molecular chaperones as modulators of polyglutamine protein aggregation and toxicity. Proc Natl Acad Sci U S A (2002) 1.65

Compositional determinants of prion formation in yeast. Mol Cell Biol (2010) 1.63

Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo. J Biol Chem (2010) 1.62

Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 2. J Neurosci (2009) 1.59

Altered transcription in yeast expressing expanded polyglutamine. Proc Natl Acad Sci U S A (2001) 1.58

Sequence composition of disordered regions fine-tunes protein half-life. Nat Struct Mol Biol (2015) 1.55

Expanded polyglutamines in Caenorhabditis elegans cause axonal abnormalities and severe dysfunction of PLM mechanosensory neurons without cell death. Proc Natl Acad Sci U S A (2001) 1.53

Proteostasis of polyglutamine varies among neurons and predicts neurodegeneration. Nat Chem Biol (2013) 1.52

The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis. Proc Natl Acad Sci U S A (2001) 1.51

Chaperoning brain degeneration. Proc Natl Acad Sci U S A (2002) 1.51

Multi-tasking on chromatin with the SAGA coactivator complexes. Mutat Res (2007) 1.50

Chemical inducers of autophagy that enhance the clearance of mutant proteins in neurodegenerative diseases. J Biol Chem (2010) 1.50

Cardiomyocyte expression of a polyglutamine preamyloid oligomer causes heart failure. Circulation (2008) 1.49

Human cytomegalovirus UL97 kinase activity is required for the hyperphosphorylation of retinoblastoma protein and inhibits the formation of nuclear aggresomes. J Virol (2008) 1.48

Ataxin 1, a SCA1 neurodegenerative disorder protein, is functionally linked to the silencing mediator of retinoid and thyroid hormone receptors. Proc Natl Acad Sci U S A (2004) 1.47

Glutamine/proline-rich PQE-1 proteins protect Caenorhabditis elegans neurons from huntingtin polyglutamine neurotoxicity. Proc Natl Acad Sci U S A (2002) 1.46

Identification of combinatorial drug regimens for treatment of Huntington's disease using Drosophila. Proc Natl Acad Sci U S A (2005) 1.45

Subcellular localisation, secretion, and post-translational processing of normal cochlin, and of mutants causing the sensorineural deafness and vestibular disorder, DFNA9. J Med Genet (2003) 1.44

Targets and consequences of protein SUMOylation in neurons. Brain Res Rev (2010) 1.44

Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 3. J Neurosci (2008) 1.44

FGF14 regulates the intrinsic excitability of cerebellar Purkinje neurons. Neurobiol Dis (2008) 1.43

Atomistic simulations of the effects of polyglutamine chain length and solvent quality on conformational equilibria and spontaneous homodimerization. J Mol Biol (2008) 1.43

Non-invasive detection of neurochemical changes prior to overt pathology in a mouse model of spinocerebellar ataxia type 1. J Neurochem (2013) 1.43

Identities of sequestered proteins in aggregates from cells with induced polyglutamine expression. J Cell Biol (2001) 1.42

Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease. Proc Natl Acad Sci U S A (2005) 1.42

Huntingtin Subcellular Localisation Is Regulated by Kinase Signalling Activity in the StHdhQ111 Model of HD. PLoS One (2015) 1.40

Pathological cell-cell interactions are necessary for striatal pathogenesis in a conditional mouse model of Huntington's disease. Mol Neurodegener (2007) 1.39

Harnessing chaperone-mediated autophagy for the selective degradation of mutant huntingtin protein. Nat Biotechnol (2010) 1.38

An expanded glutamine repeat destabilizes native ataxin-3 structure and mediates formation of parallel beta -fibrils. Proc Natl Acad Sci U S A (2001) 1.38

Monoclonal antibodies recognize distinct conformational epitopes formed by polyglutamine in a mutant huntingtin fragment. J Biol Chem (2009) 1.37

Activation of Hsp70 reduces neurotoxicity by promoting polyglutamine protein degradation. Nat Chem Biol (2012) 1.37

Physical chemistry of polyglutamine: intriguing tales of a monotonous sequence. J Mol Biol (2012) 1.37

Multiple pathways contribute to the pathogenesis of Huntington disease. Mol Neurodegener (2006) 1.35

Phosphorylation of threonine 3: implications for Huntingtin aggregation and neurotoxicity. J Biol Chem (2009) 1.34

Polyglutamine genes interact to modulate the severity and progression of neurodegeneration in Drosophila. PLoS Biol (2008) 1.33

Interruptions in the expanded ATTCT repeat of spinocerebellar ataxia type 10: repeat purity as a disease modifier? Am J Hum Genet (2005) 1.32

Polyglutamine fibrillogenesis: the pathway unfolds. Proc Natl Acad Sci U S A (2002) 1.32

Evaluating the prevalence of polyglutamine repeat expansions in amyotrophic lateral sclerosis. Neurology (2011) 1.32

Nuclear aggresomes form by fusion of PML-associated aggregates. Mol Biol Cell (2005) 1.31

De novo design of synthetic prion domains. Proc Natl Acad Sci U S A (2012) 1.30

Neurochemical alterations in spinocerebellar ataxia type 1 and their correlations with clinical status. Mov Disord (2010) 1.30

Intrabodies binding the proline-rich domains of mutant huntingtin increase its turnover and reduce neurotoxicity. J Neurosci (2008) 1.28

Flightless flies: Drosophila models of neuromuscular disease. Ann N Y Acad Sci (2010) 1.28

Intranuclear degradation of polyglutamine aggregates by the ubiquitin-proteasome system. J Biol Chem (2009) 1.26

Boat, an AXH domain protein, suppresses the cytotoxicity of mutant ataxin-1. EMBO J (2005) 1.25

Effects of intracellular expression of anti-huntingtin antibodies of various specificities on mutant huntingtin aggregation and toxicity. Proc Natl Acad Sci U S A (2002) 1.24

Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment. Proc Natl Acad Sci U S A (2002) 1.24

Altered trafficking of membrane proteins in purkinje cells of SCA1 transgenic mice. Am J Pathol (2001) 1.24

Fluorescent proteins as biomarkers and biosensors: throwing color lights on molecular and cellular processes. Curr Protein Pept Sci (2008) 1.22

Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin. J Cell Biol (2008) 1.22

Amyloid-β as a modulator of synaptic plasticity. J Alzheimers Dis (2010) 1.22

Potential aggregation prone regions in biotherapeutics: A survey of commercial monoclonal antibodies. MAbs (2009) 1.20

CAG repeat lengths > or =335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse. Neurobiol Dis (2008) 1.19

Small ubiquitin-like modifier (SUMO) modification of the androgen receptor attenuates polyglutamine-mediated aggregation. J Biol Chem (2009) 1.19

In-cell aggregation of a polyglutamine-containing chimera is a multistep process initiated by the flanking sequence. J Biol Chem (2007) 1.19

Amyloid deposits: protection against toxic protein species? Cell Cycle (2009) 1.18

RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1. Nature (2013) 1.18

Mutant huntingtin, abnormal mitochondrial dynamics, defective axonal transport of mitochondria, and selective synaptic degeneration in Huntington's disease. Biochim Biophys Acta (2011) 1.16

The interplay between PolyQ and protein context delays aggregation by forming a reservoir of protofibrils. PLoS One (2006) 1.15

Transcriptional changes in Huntington disease identified using genome-wide expression profiling and cross-platform analysis. Hum Mol Genet (2010) 1.14

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Polyglutamine-expanded androgen receptor truncation fragments activate a Bax-dependent apoptotic cascade mediated by DP5/Hrk. J Neurosci (2009) 1.10

Targeting Hsp90/Hsp70-based protein quality control for treatment of adult onset neurodegenerative diseases. Annu Rev Pharmacol Toxicol (2014) 1.09

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