Evidence map›Paper›PMID 27529157›Full record

ReviewBrain pathology (Zurich, Switzerland)2016

Huntington's disease (HD): the neuropathology of a multisystem neurodegenerative disorder of the human brain.

U Rüb, K Seidel, H Heinsen, J P Vonsattel, W F den Dunnen, H W Korf

Open access · greenAbstract readReview
In one paragraph

Review in Brain pathology (Zurich, Switzerland), 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 89 papers, 3 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
89citing papers in PubMed, 3 pooled it
6.0field-weighted citation impact, top 3% 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

89 citing papers in PubMed, 3 syntheses or guidelines pooled it, 197 citations in OpenAlex.

  1. Pooled it
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  6. Article
  7. Treatment of Huntington's disease with a pan-HTT-targeting CRISPR nuclease.Molecular therapy : the journal of the American Society of Gene Therapy · 2026
    Article
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  10. Psychosis in Huntington disease: a short update.Journal of neural transmission (Vienna, Austria : 1996) · 2026
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29 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

6 authors at 4 institutions in 4 countries.

U RübDr. Senckenbergisches Chronomedizinisches Institut, Goethe-University, Frankfurt/Main, D-60590, Germany.
K SeidelDr. Senckenbergisches Chronomedizinisches Institut, Goethe-University, Frankfurt/Main, D-60590, Germany.
H HeinsenDepartment of Pathology, University of Sao Paulo Medical School, Sao Paulo, Brazil.
J P VonsattelThe New York Brain Bank/Taub Institute, The Presbyterian Hospital and Columbia University, New York, NY.
W F den DunnenDepartment of Pathology and Medical Biology, University Medical Center Groningen University of Groningen, RB Groningen, NL-5970, The Netherlands.
H W KorfDr. Senckenbergisches Chronomedizinisches Institut, Goethe-University, Frankfurt/Main, D-60590, Germany.
Goethe University Frankfurt · DENewYork–Presbyterian Hospital · USUniversidade de São Paulo · BRUniversity Medical Center Groningen · NL

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Huntington's disease (HD) is an autosomal dominantly inherited, and currently untreatable, neuropsychiatric disorder. This progressive and ultimately fatal disease is named after the American physician George Huntington and according to the underlying molecular biological mechanisms is assigned to the human polyglutamine or CAG-repeat diseases. In the present article we give an overview of the currently known neurodegenerative hallmarks of the brains of HD patients. Subsequent to recent pathoanatomical studies the prevailing reductionistic concept of HD as a human neurodegenerative disease, which is primarily and more or less exclusively confined to the striatum (ie, caudate nucleus and putamen) has been abandoned. Many recent studies have improved our neuropathological knowledge of HD; many of the early groundbreaking findings of neuropathological HD research have been rediscovered and confirmed. The results of this investigation have led to the stepwise revision of the simplified pathoanatomical and pathophysiological HD concept and culminated in the implementation of the current concept of HD as a multisystem degenerative disease of the human brain. The multisystem character of the neuropathology of HD is emphasized by a brain distribution pattern of neurodegeneration (i) which apart from the striatum includes the cerebral neo-and allocortex, thalamus, pallidum, brainstem and cerebellum, and which (ii) therefore, shares more similarities with polyglutamine spinocerebellar ataxias than previously thought.

Indexed as

BrainGene Expression RegulationHumansHuntingtin ProteinHuntington DiseaseNeurodegenerative DiseasesPeptidesRNA-Binding ProteinsHTT protein, humanHuntingtin ProteinP62 protein, humanPeptidespolyglutamineRNA-Binding Proteinsbrainstemcerebellumcerebral cortexpathoanatomypolyglutamine diseasesthalamus

Identifiers

PMID27529157
PMCPMC8029421
OpenAlexW2513968848

What OpenQuestion holds

Textmetadata
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.