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Joana M. Gil et al.
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JOURNAL OF NEUROSCIENCE RESEARCH (2009)
Omi/HtrA2 is relevant to the selective vulnerability of striatal neurons in Huntington's disease
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Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1
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TDP1 facilitates chromosomal single-strand break repair in neurons and is neuroprotective in vivo
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EMBO JOURNAL (2007)
Global changes to the ubiquitin system in Huntington's disease
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Senataxin, defective in ataxia oculomotor apraxia type 2, is involved in the defense against oxidative DNA damage
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HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS
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OGG1 initiates age-dependent CAG trinucleotide expansion in somatic cells
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Ku70/80 modulates ATM and ATR signaling pathways in response to DNA double strand breaks
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JOURNAL OF BIOLOGICAL CHEMISTRY (2007)
Proteome analysis of soluble nuclear proteins reveals that HMGB1/2 suppress genotoxic stress in polyglutamine diseases
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Distinct faces of the Ku heterodimer mediate DNA repair and telomeric functions
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Characterization of neuron-specific huntingtin aggregates in human huntingtin knock-in mice
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NEUROSCIENCE RESEARCH (2007)
The induction levels of heat shock protein 70 differentiate the vulnerabilities to mutant huntingtin among neuronal subtypes
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ATAXIN-1 interacts with the repressor capicua in its native complex to cause SCA1 neuropathology
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Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1α in Huntington's disease neurodegeneration
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Transcriptional repression of PGC-α by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration
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Cell apoptosis: Requirement of H2AX in DNA ladder formation, but not for the activation of caspase-3
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Selective utilization of nonhomologous end-joining and homologous recombination DNA repair pathways during nervous system development
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A topoisomerase IIβ-mediated dsDNA break required for regulated transcription
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Spatial organization of the mammalian genome surveillance machinery in response to DNA strand breaks
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JOURNAL OF CELL BIOLOGY (2006)
Transcriptional repression induces a slowly progressive atypical neuronal death associated with changes of YAP isoforms and p73
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JOURNAL OF CELL BIOLOGY (2006)
The comet assay: a method to measure DNA damage in individual cells
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HMG proteins: dynamic players in gene regulation and differentiation
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CURRENT OPINION IN GENETICS & DEVELOPMENT (2005)
Therapeutics development for triplet repeat expansion diseases
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NATURE REVIEWS GENETICS (2005)
Increased susceptibility of cytoplasmic over nuclear polyglutamine aggregates to autophagic degradation
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PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2005)
p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease
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NEURON (2005)
A genomic screen in yeast implicates kynurenine 3-monooxygenase as a therapeutic target for Huntington disease
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SCIENCE (2005)
Defective DNA single-strand break repair in spinocerebellar ataxia with axonal neuropathy-1
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Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins
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ATM and DNA-PK function redundantly to phosphorylate H2AX after exposure to ionizing radiation
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CANCER RESEARCH (2004)
Striatal cells from mutant huntingtin knock-in mice are selectively vulnerable to mitochondrial complex II inhibitor-induced cell death through a non-apoptotic pathway
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HUMAN MOLECULAR GENETICS (2004)
Acetylation of the C terminus of Ku70 by CBP and PCAF controls Bax-mediated apoptosis
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MOLECULAR CELL (2004)
A protein interaction network links GIT1, an enhancer of huntingtin aggregation, to Huntington's disease
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H2AX: the histone guardian of the genome
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Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules
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Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease
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NATURE GENETICS (2004)
Enhanced striatal NR2B-containing N-methyl-D-aspartate receptor-mediated synaptic currents in a mouse model of Huntington disease
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DNA end resection, homologous recombination and DNA damage checkpoint activation require CDK1
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Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death
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Distinct aggregation and cell death patterns among different types of primary neurons induced by mutant huntingtin protein
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HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis
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Telomere length deregulation and enhanced sensitivity to genotoxic stress in Arabidopsis mutants deficient in Ku70
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Interaction between mutant ataxin-1 and POBP-1 affects transcription and cell death
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Heat shock protein 27 prevents cellular polyglutamine toxicity and suppresses the increase of reactive oxygen species caused by huntingtin
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HUMAN MOLECULAR GENETICS (2002)
HIP1 functions in clathrin-mediated endocytosis through binding to clathrin and adaptor protein 2
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Early-onset ataxia with ocular motor apraxia and hypoalbuminemia is caused by mutations in a new HIT superfamily gene
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NATURE GENETICS (2001)
Age-dependent and tissue-specific CAG repeat instability occurs in mouse knock-in for a mutant Huntington's disease gene
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Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease
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HUMAN MOLECULAR GENETICS (2001)
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p53 Binding protein 1 (53BP1) is an early participant in the cellular response to DNA double-strand breaks
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Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity
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PQBP-1/Npw38, a nuclear protein binding to the polyglutamine tract, interacts with US-15kD/dim1p via the carboxyl-terminal domain
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The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription
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