Evidence map›Paper›PMID 31353533›Full record

ReviewJournal of neuroscience research2019

Developmental origins of cortical hyperexcitability in Huntington's disease: Review and new observations.

Carlos Cepeda, Katerina D Oikonomou, Damian Cummings, Joshua Barry, Vannah-Wila Yazon, Dickson T Chen, Janelle Asai, Christopher K Williams, Harry V Vinters

Abstract readReview
PubMed Full text at PMC Publisher
In one paragraph

Review in Journal of neuroscience research, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.

0numbers the graph read from it
0cells of the map it votes in
18citing papers in PubMed
–field-weighted citation impact
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

18 citing papers in PubMed.

  1. Trial
  2. Article
  3. Article
  4. Article
  5. Review
  6. Article
  7. Article
  8. Article
  9. Review
  10. Review
  11. Article
  12. Synaptic Dysfunction in Huntington's Disease: Lessons from Genetic Animal Models.The Neuroscientist : a review journal bringing neurobiology, neurology and psychiatry · 2022
    Article
  13. Review
  14. Article
  15. Juvenile-Onset Huntington Disease Pathophysiology and Neurodevelopment: A Review.Movement disorders : official journal of the Movement Disorder Society · 2022
    Review
  16. Review
  17. Article
  18. The Neurodevelopmental Hypothesis of Huntington's Disease.Journal of Huntington's disease · 2020
    Review
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

9 authors.

Carlos CepedaIDDRC, Jane and Terry Semel Institute for Neuroscience and Human Behavior, University of California Los Angeles, Los Angeles, California.ORCID 0000-0001-5953-6692
Katerina D OikonomouIDDRC, Jane and Terry Semel Institute for Neuroscience and Human Behavior, University of California Los Angeles, Los Angeles, California.
Damian CummingsIDDRC, Jane and Terry Semel Institute for Neuroscience and Human Behavior, University of California Los Angeles, Los Angeles, California.ORCID 0000-0001-5567-0907
Joshua BarryIDDRC, Jane and Terry Semel Institute for Neuroscience and Human Behavior, University of California Los Angeles, Los Angeles, California.
Vannah-Wila YazonIDDRC, Jane and Terry Semel Institute for Neuroscience and Human Behavior, University of California Los Angeles, Los Angeles, California.
Dickson T ChenIDDRC, Jane and Terry Semel Institute for Neuroscience and Human Behavior, University of California Los Angeles, Los Angeles, California.
Janelle AsaiIDDRC, Jane and Terry Semel Institute for Neuroscience and Human Behavior, University of California Los Angeles, Los Angeles, California.
Christopher K WilliamsSection of Neuropathology, Department of Pathology and Laboratory Medicine, University of California Los Angeles, Los Angeles, California.
Harry V VintersSection of Neuropathology, Department of Pathology and Laboratory Medicine, University of California Los Angeles, Los Angeles, California.

Funding

Cortical Maldevelopment Contributes to the Pathophysiology of Huntington’s DiseaseR21NS111316 · NINDS · UNIVERSITY OF CALIFORNIA LOS ANGELES · PI CEPEDA, CARLOS T · 2019 to 2019
$429k
NINDS NIH HHS R21 NS111316U.S. Public Health Service NS111316
6 · The paper itself

Abstract

Huntington's disease (HD), an inherited neurodegenerative disorder that principally affects striatum and cerebral cortex, is generally thought to have an adult onset. However, a small percentage of cases develop symptoms before 20 years of age. This juvenile variant suggests that brain development may be altered in HD. Indeed, recent evidence supports an important role of normal huntingtin during embryonic brain development and mutations in this protein cause cortical abnormalities. Functional studies also demonstrated that the cerebral cortex becomes hyperexcitable with disease progression. In this review, we examine clinical and experimental evidence that cortical development is altered in HD. We also provide preliminary evidence that cortical pyramidal neurons from R6/2 mice, a model of juvenile HD, are hyperexcitable and display dysmorphic processes as early as postnatal day 7. Further, some symptomatic mice present with anatomical abnormalities reminiscent of human focal cortical dysplasia, which could explain the occurrence of epileptic seizures in this genetic mouse model and in children with juvenile HD. Finally, we discuss recent treatments aimed at correcting abnormal brain development.

Indexed as

Cortical ExcitabilityAnimalsCerebral CortexDisease Models, AnimalHumansHuntingtin ProteinHuntington DiseaseMice, TransgenicNeuronsHTT protein, humanHuntingtin Proteincortical developmentcortical dysplasiaelectrophysiologyepilepsyHuntington's disease

Identifiers

PMID31353533
PMCPMC6801077

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Registered trials

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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.