Evidence map›Paper›PMID 28384474›Full record

ArticleNeuron2017

Polyglutamine-Expanded Huntingtin Exacerbates Age-Related Disruption of Nuclear Integrity and Nucleocytoplasmic Transport.

Fatima Gasset-Rosa, Carlos Chillon-Marinas, Alexander Goginashvili, Ranjit Singh Atwal, Jonathan W Artates, Ricardos Tabet, Vanessa C Wheeler, Anne G Bang, Don W Cleveland, Clotilde Lagier-Tourenne

Open access · bronzeAbstract readVideo-Audio Media
PubMed Full text at PMC Publisher
In one paragraph

Article in Neuron, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 150 papers.

0numbers the graph read from it
0cells of the map it votes in
150citing papers in PubMed
13.4field-weighted citation impact, top 1% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

150 citing papers in PubMed, 247 citations in OpenAlex.

  1. Article
  2. Article
  3. Review
  4. Nuclear dysfunction in aging and neurodegeneration.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2026
    Review
  5. Review
  6. Article
  7. Article
  8. Article
  9. Article
  10. Article
  11. Article
  12. Article
  13. Connecting the Dots: Stress Granule and Cardiovascular Diseases.Journal of cardiovascular translational research · 2025
    Review
  14. Proteostasis and Its Role in Disease Development.Cell biochemistry and biophysics · 2025
    Review
  15. Article
  16. Article
  17. Review
  18. Review
  19. Article
  20. Review

90 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

10 authors at 6 institutions in 1 country.

Fatima Gasset-RosaLudwig Institute for Cancer Research, University of California at San Diego, La Jolla, CA 92093, USA; Department of Cellular and Molecular Medicine, University of California at San Diego, La Jolla, CA 92093, USA.
Carlos Chillon-MarinasLudwig Institute for Cancer Research, University of California at San Diego, La Jolla, CA 92093, USA; Department of Cellular and Molecular Medicine, University of California at San Diego, La Jolla, CA 92093, USA.
Alexander GoginashviliLudwig Institute for Cancer Research, University of California at San Diego, La Jolla, CA 92093, USA; Department of Cellular and Molecular Medicine, University of California at San Diego, La Jolla, CA 92093, USA.
Ranjit Singh AtwalDepartment of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA; Center for Genomic Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.
Jonathan W ArtatesLudwig Institute for Cancer Research, University of California at San Diego, La Jolla, CA 92093, USA; Department of Cellular and Molecular Medicine, University of California at San Diego, La Jolla, CA 92093, USA.
Ricardos TabetDepartment of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA; Broad Institute of Harvard University and MIT, Cambridge, MA 02142, USA.
Vanessa C WheelerDepartment of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA; Center for Genomic Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.
Anne G BangConrad Prebys Center for Chemical Genomics, Sanford Burnham Prebys Medical Discovery Institute, La Jolla, CA 92037, USA.
Don W ClevelandLudwig Institute for Cancer Research, University of California at San Diego, La Jolla, CA 92093, USA; Department of Cellular and Molecular Medicine, University of California at San Diego, La Jolla, CA 92093, USA. Electronic address: dcleveland@ucsd.edu.
Clotilde Lagier-TourenneDepartment of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA; Broad Institute of Harvard University and MIT, Cambridge, MA 02142, USA. Electronic address: clagier-tourenne@mgh.harvard.edu.
University of California, San Diego · USHarvard University · USLudwig Cancer Research · USBroad Institute · USDiscovery Institute · USMassachusetts General Hospital · US

Funding

VIRUS-INDUCED BRAIN PATHOLOGYP50AG005131 · NIA · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI BREWER, JAMES B · 1985 to 2018
$55.4M
THE ROLE OF ADVANCED CAA IN ALZHEIMER'S DEMENTIAP50AG005134 · NIA · MASSACHUSETTS GENERAL HOSPITAL · PI HYMAN, BRADLEY T. · 1985 to 2018
$42.1M
Using RNA signatures for therapy development in neurodegeneration due to C9orf72 expansionsR01NS087227 · NINDS · MASSACHUSETTS GENERAL HOSPITAL · PI LAGIER-TOURENNE, CLOTILDE · 2014 to 2018
$1.5M
NIA NIH HHS P50 AG005131NIA NIH HHS P50 AG005134NINDS NIH HHS R01 NS087227
6 · The paper itself

Abstract

Onset of neurodegenerative disorders, including Huntington's disease, is strongly influenced by aging. Hallmarks of aged cells include compromised nuclear envelope integrity, impaired nucleocytoplasmic transport, and accumulation of DNA double-strand breaks. We show that mutant huntingtin markedly accelerates all of these cellular phenotypes in a dose- and age-dependent manner in cortex and striatum of mice. Huntingtin-linked polyglutamine initially accumulates in nuclei, leading to disruption of nuclear envelope architecture, partial sequestration of factors essential for nucleocytoplasmic transport (Gle1 and RanGAP1), and intranuclear accumulation of mRNA. In aged mice, accumulation of RanGAP1 together with polyglutamine is shifted to perinuclear and cytoplasmic areas. Consistent with findings in mice, marked alterations in nuclear envelope morphology, abnormal localization of RanGAP1, and nuclear accumulation of mRNA were found in cortex of Huntington's disease patients. Overall, our findings identify polyglutamine-dependent inhibition of nucleocytoplasmic transport and alteration of nuclear integrity as a central component of Huntington's disease.

Indexed as

Active Transport, Cell NucleusAdultAged, 80 and overAgingAnimalsCase-Control StudiesCell NucleusCerebral CortexFemaleGTPase-Activating ProteinsHumansHuntingtin ProteinMaleMiceMiddle AgedMutationGle1 protein, humanGle1 protein, mouseGTPase-Activating ProteinsHTT protein, humanHtt protein, mouseHuntingtin ProteinNucleocytoplasmic Transport ProteinsPeptidespolyglutamineRANGAP1 protein, humanRangap1 protein, mouseRNA, Messenger

Identifiers

PMID28384474
PMCPMC5479704
OpenAlexW2605283453

What OpenQuestion holds

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Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.