Evidence map›Paper›PMID 25982834›Full record

ReviewNeurobiology of disease2016

Unexpected cellular players in Rett syndrome pathology.

James C Cronk, Noel C Derecki, Vladimir Litvak, Jonathan Kipnis

Open access · greenAbstract readReview
In one paragraph

Review in Neurobiology of disease, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.

0numbers the graph read from it
0cells of the map it votes in
14citing papers in PubMed
4.2field-weighted citation impact, top 6% 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

14 citing papers in PubMed, 32 citations in OpenAlex.

  1. Article
  2. Atypical case of Rett syndrome with concurrentWorld journal of clinical pediatrics · 2025
    Article
  3. Rett and Rett-related disorders: Common mechanisms for shared symptoms?Experimental biology and medicine (Maywood, N.J.) · 2023
    Review
  4. Natural Course ofChildren (Basel, Switzerland) · 2023
    Article
  5. State-of-the-art therapies for Rett syndrome.Developmental medicine and child neurology · 2023
    Review
  6. Article
  7. Review
  8. DNA Methylation-Governed Gene Expression in Autoimmune Arthritis.International journal of molecular sciences · 2019
    Review
  9. Review
  10. Article
  11. Article
  12. New "programmers" in tissue macrophage activation.Pflugers Archiv : European journal of physiology · 2017
    Review
  13. CXBrain, behavior, and immunity · 2017
    Article
  14. Article
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

4 authors at 3 institutions in 1 country.

James C CronkCenter for Brain Immunology and Glia, Department of Neuroscience, Graduate Program in Neuroscience and Medical Scientist Training Program, University of Virginia, Charlottesville, VA 22908, USA. Electronic address: jcc2cm@virginia.edu.
Noel C DereckiCenter for Brain Immunology and Glia, Department of Neuroscience, Graduate Program in Neuroscience and Medical Scientist Training Program, University of Virginia, Charlottesville, VA 22908, USA.
Vladimir LitvakDepartment of Microbiology and Physiological Systems, University of Massachusetts Medical School, Worcester, MA 01655, USA.
Jonathan KipnisCenter for Brain Immunology and Glia, Department of Neuroscience, Graduate Program in Neuroscience and Medical Scientist Training Program, University of Virginia, Charlottesville, VA 22908, USA. Electronic address: kipnis@virginia.edu.
University of Virginia Medical Center · USUniversity of Massachusetts Chan Medical School · USUniversity of Virginia · US

Funding

MEDICAL SCIENTIST TRAINING PROGRAMT32GM007267 · NIGMS · UNIVERSITY OF VIRGINIA CHARLOTTESVILLE · PI GARCIA-BLANCO, MARIANO A. · 1985 to 2024
$13.4M
Innate immunity in Rett pathology and repairR01NS081026 · NINDS · UNIVERSITY OF VIRGINIA · PI KIPNIS, JONATHAN · 2012 to 2016
$1.7M
Immune pathology and bone marrow transplant in disorders of Mecp2 overexpressionF30AI109984 · NIAID · UNIVERSITY OF VIRGINIA · PI CRONK, JAMES CHRISTOPHER · 2014 to 2017
$152k
NIAID NIH HHS F30 AI109984NIGMS NIH HHS T32 GM007267NINDS NIH HHS R01 NS081026
6 · The paper itself

Abstract

Rett syndrome is a devastating neurodevelopmental disorder, primarily caused by mutations of methyl CpG-binding protein 2 (MeCP2). Although the genetic cause of disease was identified over a decade ago, a significant gap still remains in both our clinical and scientific understanding of its pathogenesis. Neurons are known to be primary players in pathology, with their dysfunction being the key in Rett syndrome. While studies in mice have demonstrated a clear causative - and potential therapeutic - role for neurons in Rett syndrome, recent work has suggested that other tissues also contribute significantly to progression of the disease. Indeed, Rett syndrome is known to present with several common peripheral pathologies, such as osteopenia, scoliosis, gastrointestinal problems including nutritional defects, and general growth deficit. Mouse models assessing the potential role of non-neuronal cell types have confirmed both roles in disease and potential therapeutic targets. A new picture is emerging in which neurons both initiate and drive pathology, while dysfunction of other cell types and peripheral tissues exacerbate disease, possibly amplifying further neurologic problems, and ultimately result in a positive feedback loop of progressively worsening symptoms. Here, we review what is known about neuronal and non-neuronal cell types, and discuss how this new, integrative understanding of the disease may allow for additional clinical and scientific pathways for treating and understanding Rett syndrome.

Indexed as

AnimalsHumansRett Syndrome

Identifiers

PMID25982834
PMCPMC4644494
OpenAlexW323936931

What OpenQuestion holds

Textmetadata
LicenceTDM
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.