(Q38925490)

7 September 2017

title

Targeting ATM ameliorates mutant Huntingtin toxicity in cell and animal models of Huntington's disease (English)

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7 September 2017

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Henry Waldvogel

7

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Richard Faull

object named as

Richard Faull

15

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Harry V Vinters

14

object named as

Harry V Vinters

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7 September 2017

Xiao-Hong Lu

1

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7 September 2017

Virginia B Mattis

2

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7 September 2017

Nan Wang

3

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7 September 2017

Ismael Al-Ramahi

4

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7 September 2017

Nick van den Berg

5

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7 September 2017

Silvina A Fratantoni

6

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7 September 2017

Erin Greiner

8

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7 September 2017

Alex Osmand

9

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7 September 2017

Karla Elzein

10

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7 September 2017

Jingbo Xiao

11

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7 September 2017

Sipke Dijkstra

12

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7 September 2017

Remko de Pril

13

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7 September 2017

Ethan Signer

16

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7 September 2017

Seung Kwak

17

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7 September 2017

Juan J Marugan

18

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7 September 2017

Juan Botas

19

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7 September 2017

David F Fischer

20

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7 September 2017

Clive N Svendsen

21

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7 September 2017

Ignacio Munoz-Sanjuan

22

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7 September 2017

X William Yang

23

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7 September 2017

language of work or name

English

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publication date

1 December 2014

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Science Translational Medicine

7 September 2017

published in

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7 September 2017

volume

6

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7 September 2017

issue

268

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7 September 2017

page(s)

268ra178

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https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

7 September 2017

1 reference

Huntington's disease: from molecular pathogenesis to clinical treatment

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Oxidative damage in Huntington's disease pathogenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Network organization of the huntingtin proteomic interactome in mammalian brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Elevated NADPH oxidase activity contributes to oxidative stress and cell death in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Glutathione peroxidase activity is neuroprotective in models of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

DNA repair, genome stability, and aging

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

The ATM protein kinase: regulating the cellular response to genotoxic stress, and more

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

ATM and ataxia telangiectasia

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

The ATM protein kinase and cellular redox signaling: beyond the DNA damage response

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

The DNA Damage Response: Making It Safe to Play with Knives

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

ATM-dependent chromatin changes silence transcription in cis to DNA double-strand breaks

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Heterozygous ATM mutations do not contribute to early onset of breast cancer

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Atm-deficient mice: a paradigm of ataxia telangiectasia

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Mice heterozygous for mutation in Atm, the gene involved in ataxia-telangiectasia, have heightened susceptibility to cancer.

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Requirement for Atm in ionizing radiation-induced cell death in the developing central nervous system

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Cell cycle activation linked to neuronal cell death initiated by DNA damage

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Reduced NMDA-induced apoptosis in neurons lacking ataxia telangiectasia mutated protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Physiologic brain activity causes DNA double-strand breaks in neurons, with exacerbation by amyloid-β

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

DNA breakage and induction of DNA damage response proteins precede the appearance of visible mutant huntingtin aggregates

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

DNA damage induced by polyglutamine-expanded proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Early and late events induced by polyQ-expanded proteins: identification of a common pathogenic property of polYQ-expanded proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Mutant huntingtin impairs Ku70-mediated DNA repair

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Mutations of the Huntington's disease protein impact on the ATM-dependent signaling and repair pathways of the radiation-induced DNA double-strand breaks: corrective effect of statins and bisphosphonates.

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

IKKalpha and IKKbeta regulation of DNA damage-induced cleavage of huntingtin

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Induced pluripotent stem cells from patients with Huntington's disease show CAG-repeat-expansion-associated phenotypes

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Huntingtin interacting proteins are genetic modifiers of neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Drosophila atm/telomere fusion is required for telomeric localization of HP1 and telomere position effect.

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Behavioral phenotyping of transgenic and knockout mice: experimental design and evaluation of general health, sensory functions, motor abilities, and specific behavioral tests

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Neuronal targets for reducing mutant huntingtin expression to ameliorate disease in a mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Centrosome disorganization in fibroblast cultures derived from R6/2 Huntington's disease (HD) transgenic mice and HD patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Identification and characterization of a novel and specific inhibitor of the ataxia-telangiectasia mutated kinase ATM

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Improved ATM kinase inhibitor KU-60019 radiosensitizes glioma cells, compromises insulin, AKT and ERK prosurvival signaling, and inhibits migration and invasion

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Composite primary neuronal high-content screening assay for Huntington's disease incorporating non-cell-autonomous interactions

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Phosphorylation of huntingtin by cyclin-dependent kinase 5 is induced by DNA damage and regulates wild-type and mutant huntingtin toxicity in neurons.

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Deregulation of BRCA1 leads to impaired spatiotemporal dynamics of γ-H2AX and DNA damage responses in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Neuroinflammation in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

ATM activation by oxidative stress

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Cystathionine γ-lyase deficiency mediates neurodegeneration in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

ATM and ATR substrate analysis reveals extensive protein networks responsive to DNA damage

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Nuclear accumulation of HDAC4 in ATM deficiency promotes neurodegeneration in ataxia telangiectasia

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

EZH2-mediated H3K27 trimethylation mediates neurodegeneration in ataxia-telangiectasia

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

SIRT1 collaborates with ATM and HDAC1 to maintain genomic stability in neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Neuroprotective role of Sirt1 in mammalian models of Huntington's disease through activation of multiple Sirt1 targets

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Sirt1 mediates neuroprotection from mutant huntingtin by activation of the TORC1 and CREB transcriptional pathway

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

HDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Huntingtin facilitates polycomb repressive complex 2.

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

High-content chemical and RNAi screens for suppressors of neurotoxicity in a Huntington's disease model

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Huntington's disease is a disorder of the corpus striatum: focus on Rhes (Ras homologue enriched in the striatum).

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Cytoplasmic ATM in neurons modulates synaptic function

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Caffeine inhibits the checkpoint kinase ATM

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Neuroprotective effects of caffeine against complex I inhibition-induced apoptosis are mediated by inhibition of the Atm/p53/E2F-1 path in cerebellar granule neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

EZ spheres: a stable and expandable culture system for the generation of pre-rosette multipotent stem cells from human ESCs and iPSCs

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Progressive incorporation of propidium iodide in cultured mouse neurons correlates with declining electrophysiological status: a fluorescence scale of membrane integrity

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Mutant huntingtin forms in vivo complexes with distinct context-dependent conformations of the polyglutamine segment

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

A single short stretch of homology between adenoviral vector and packaging cell line can give rise to cytopathic effect-inducing, helper-dependent E1-positive particles

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Quantification assays for total and polyglutamine-expanded huntingtin proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

Single-step detection of mutant huntingtin in animal and human tissues: a bioassay for Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3010523

10.1126/SCITRANSLMED.3010523

21 January 2018

Identifiers

DOI

1 reference

7 September 2017

1 reference