Senataxin, defective in ataxia oculomotor apraxia type 2, is involved in the defense against oxidative DNA damage
about
Out of balance: R-loops in human diseaseCK2 phosphorylation-dependent interaction between aprataxin and MDC1 in the DNA damage responseHuman senataxin resolves RNA/DNA hybrids formed at transcriptional pause sites to promote Xrn2-dependent terminationProtein interaction analysis of senataxin and the ALS4 L389S mutant yields insights into senataxin post-translational modification and uncovers mutant-specific binding with a brain cytoplasmic RNA-encoded peptideA role for DEAD box 1 at DNA double-strand breaksFunctional role for senataxin, defective in ataxia oculomotor apraxia type 2, in transcriptional regulationA SUMO-dependent interaction between Senataxin and the exosome, disrupted in the neurodegenerative disease AOA2, targets the exosome to sites of transcription-induced DNA damageRecent advances in understanding transcription termination by RNA polymerase IIR-loops in proliferating cells but not in the brain: implications for AOA2 and other autosomal recessive ataxiasOxr1 is essential for protection against oxidative stress-induced neurodegenerationThe Ighmbp2 helicase structure reveals the molecular basis for disease-causing mutations in DMSA1A bacterial-like mechanism for transcription termination by the Sen1p helicase in budding yeast.Sen1p contributes to genomic integrity by regulating expression of ribonucleotide reductase 1 (RNR1) in Saccharomyces cerevisiae.Senataxin associates with replication forks to protect fork integrity across RNA-polymerase-II-transcribed genes.Transcription termination by nuclear RNA polymerasesSenataxin, defective in the neurodegenerative disorder ataxia with oculomotor apraxia 2, lies at the interface of transcription and the DNA damage response.Senataxin suppresses the antiviral transcriptional response and controls viral biogenesisA novel c.5308_5311delGAGA mutation in Senataxin in a Cypriot family with an autosomal recessive cerebellar ataxia.Novel mutations in ataxia telangiectasia and AOA2 associated with prolonged survivalSen1p performs two genetically separable functions in transcription and processing of U5 small nuclear RNA in Saccharomyces cerevisiaeClinical and molecular characterization of ataxia with oculomotor apraxia patients in Saudi Arabia.Mutant huntingtin impairs Ku70-mediated DNA repair.Ataxia with oculomotor apraxia type 2 fibroblasts exhibit increased susceptibility to oxidative DNA damageMutation of senataxin alters disease-specific transcriptional networks in patients with ataxia with oculomotor apraxia type 2Ku70 alleviates neurodegeneration in Drosophila models of Huntington's diseaseYeast Sen1 helicase protects the genome from transcription-associated instability.(1)H MR spectroscopy in Friedreich's ataxia and ataxia with oculomotor apraxia type 2.RNA helicases in infection and disease.Saccharomyces cerevisiae Sen1 as a model for the study of mutations in human Senataxin that elicit cerebellar ataxia.Genes and genetic testing in hereditary ataxias.Mutations in the selenocysteine insertion sequence-binding protein 2 gene lead to a multisystem selenoprotein deficiency disorder in humans.Clinical and genetic diversity of SMN1-negative proximal spinal muscular atrophiesSenataxin plays an essential role with DNA damage response proteins in meiotic recombination and gene silencingTransgenic animal models of neurodegeneration based on human genetic studies.Purkinje cell degeneration in pcd mice reveals large scale chromatin reorganization and gene silencing linked to defective DNA repairXRN2 Links Transcription Termination to DNA Damage and Replication Stress.A new model to study neurodegeneration in ataxia oculomotor apraxia type 2Dynamic monitoring of oxidative DNA double-strand break and repair in cardiomyocytes.The involvement of DNA-damage and -repair defects in neurological dysfunctionDNA damage and repair: relevance to mechanisms of neurodegeneration
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P2860
Senataxin, defective in ataxia oculomotor apraxia type 2, is involved in the defense against oxidative DNA damage
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2007 nî lūn-bûn
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2007 թուականի Յունիսին հրատարակուած գիտական յօդուած
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2007 թվականի հունիսին հրատարակված գիտական հոդված
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2007年の論文
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2007年論文
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2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
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2007年论文
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Senataxin, defective in ataxia ...... e against oxidative DNA damage
@ast
Senataxin, defective in ataxia ...... e against oxidative DNA damage
@en
Senataxin, defective in ataxia ...... e against oxidative DNA damage
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Senataxin, defective in ataxia ...... e against oxidative DNA damage
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Senataxin, defective in ataxia ...... e against oxidative DNA damage
@ast
Senataxin, defective in ataxia ...... e against oxidative DNA damage
@en
Senataxin, defective in ataxia ...... e against oxidative DNA damage
@en-gb
Senataxin, defective in ataxia ...... e against oxidative DNA damage
@nl
prefLabel
Senataxin, defective in ataxia ...... e against oxidative DNA damage
@ast
Senataxin, defective in ataxia ...... e against oxidative DNA damage
@en
Senataxin, defective in ataxia ...... e against oxidative DNA damage
@en-gb
Senataxin, defective in ataxia ...... e against oxidative DNA damage
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P2093
P2860
P921
P3181
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P1476
Senataxin, defective in ataxia ...... e against oxidative DNA damage
@en
P2093
Alessandro Filla
Chiara Criscuolo
Jun Nakamura
Luciana Chessa
Magtouf Gatei
Markus Fusser
Martin F Lavin
Natalie Rundle
Nuri Gueven
P2860
P304
P3181
P356
10.1083/JCB.200701042
P407
P577
2007-06-11T00:00:00Z