Evidence map›Paper›PMID 30502397›Full record

ReviewNeurobiology of learning and memory2019

Altered trajectories of neurodevelopment and behavior in mouse models of Rett syndrome.

Elizabeth S Smith, Dani R Smith, Charlotte Eyring, Maria Braileanu, Karen S Smith-Connor, Yew Ei Tan, Amanda Y Fowler, Gloria E Hoffman, Michael V Johnston, Sujatha Kannan and 1 more

Open access · greenAbstract readReview
PubMed Full text at PMC Publisher
In one paragraph

Review in Neurobiology of learning and memory, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.

0numbers the graph read from it
0cells of the map it votes in
10citing papers in PubMed
1.6field-weighted citation impact, top 14% 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

10 citing papers in PubMed, 16 citations in OpenAlex.

  1. Article
  2. Epigenetic regulation of brain development, plasticity, and response to early-life stress.Neuropsychopharmacology : official publication of the American College of Neuropsychopharmacology · 2026
    Review
  3. Article
  4. Article
  5. Review
  6. Review
  7. Review
  8. Article
  9. Rett Syndrome and CDKL5 Deficiency Disorder: From Bench to Clinic.International journal of molecular sciences · 2019
    Review
  10. 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

11 authors at 4 institutions in 2 countries.

Elizabeth S SmithDepartment of Anesthesiology and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.
Dani R SmithDepartment of Psychological and Brain Sciences, Johns Hopkins University, Baltimore, MD 21218, USA.
Charlotte EyringThe Hugo W. Moser Research Institute at Kennedy Krieger, Inc., Baltimore, MD 21205, USA.
Maria BraileanuUndergraduate Program in Neuroscience, The Krieger School of Arts and Sciences, Johns Hopkins University, Baltimore, MD 21218, USA.
Karen S Smith-ConnorThe Hugo W. Moser Research Institute at Kennedy Krieger, Inc., Baltimore, MD 21205, USA.
Yew Ei TanPerdana University Graduate School of Medicine, Kuala Lumpur, Malaysia.
Amanda Y FowlerDepartment of Biology, Morgan State University, Baltimore, MD 21251, USA.
Gloria E HoffmanDepartment of Biology, Morgan State University, Baltimore, MD 21251, USA.
Michael V JohnstonDepartment of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA; Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA; The Hugo W. Moser Research Institute at Kennedy Krieger, Inc., Baltimore, MD 21205, USA.
Sujatha KannanDepartment of Anesthesiology and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA; Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA; The Hugo W. Moser Research Institute at Kennedy Krieger, Inc., Baltimore, MD 21205, USA.
Mary E BlueDepartment of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA; The Solomon H. Snyder Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA; The Hugo W. Moser Research Institute at Kennedy Krieger, Inc., Baltimore, MD 21205, USA. Electronic address: blue@kennedykrieger.org.
Johns Hopkins University · USKennedy Krieger Institute · USMorgan State University · USPerdana University · MY

Funding

Translational Neuroscience CoreU54HD079123 · NICHD · HUGO W. MOSER RES INST KENNEDY KRIEGER · PI FATEMI, S. ALI, SCHLAGGAR, BRADLEY L · 2014 to 2019
$7.9M
Targeted Therapies for Rett SyndromeR01NS113140 · NINDS · JOHNS HOPKINS UNIVERSITY · PI KANNAN, SUJATHA · 2019 to 2023
$2.0M
Dendrimer therapies for treatment of Rett SyndromeR21NS100085 · NINDS · JOHNS HOPKINS UNIVERSITY · PI BLUE, MARY E, KANNAN, SUJATHA · 2016 to 2017
$464k
Carl Zeiss AxioImager Z1 Research MicroscopeS10RR027445 · NCRR · HUGO W. MOSER RES INST KENNEDY KRIEGER · PI BLUE, MARY E · 2010 to 2010
$139k
K+ CHANNELS IN CAROTID BODY CELLS AND GNRHF32NS010085 · NINDS · NEW YORK UNIVERSITY SCHOOL OF MEDICINE · PI SPERGEL, DANIEL J · 1995 to 1995
–
NCRR NIH HHS S10 RR027445NICHD NIH HHS U54 HD079123NINDS NIH HHS F32 NS010085NINDS NIH HHS R01 NS113140NINDS NIH HHS R21 NS100085
6 · The paper itself

Abstract

Rett Syndrome (RTT) is a genetic disorder that is caused by mutations in the x-linked gene coding for methyl-CpG-biding-protein 2 (MECP2) and that mainly affects females. Male and female transgenic mouse models of RTT have been studied extensively, and we have learned a great deal regarding RTT neuropathology and how MeCP2 deficiency may be influencing brain function and maturation. In this manuscript we review what is known concerning structural and coinciding functional and behavioral deficits in RTT and in mouse models of MeCP2 deficiency. We also introduce our own corroborating data regarding behavioral phenotype and morphological alterations in volume of the cortex and striatum and the density of neurons, aberrations in experience-dependent plasticity within the barrel cortex and the impact of MeCP2 loss on glial structure. We conclude that regional structural changes in genetic models of RTT show great similarity to the alterations in brain structure of patients with RTT. These region-specific modifications often coincide with phenotype onset and contribute to larger issues of circuit connectivity, progression, and severity. Although the alterations seen in mouse models of RTT appear to be primarily due to cell-autonomous effects, there are also non-cell autonomous mechanisms including those caused by MeCP2-deficient glia that negatively impact healthy neuronal function. Collectively, this body of work has provided a solid foundation on which to continue to build our understanding of the role of MeCP2 on neuronal and glial structure and function, its greater impact on neural development, and potential new therapeutic avenues.

Indexed as

AnimalsBasal GangliaBrainDisease Models, AnimalHippocampusHumansMethyl-CpG-Binding Protein 2MiceMotor DisordersNeuronal PlasticityRett SyndromeMecp2 protein, mouseMethyl-CpG-Binding Protein 2Barrel fieldBehaviorDevelopmental disorderGlutamate receptors, reviewMeCP2Molecular biologyMorphometryNeuropathologyPlasticityRett

Identifiers

PMID30502397
PMCPMC8040058
OpenAlexW2903199583

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.