Evidence map›Paper›PMID 33867523›Full record

ArticleMolecular psychiatry2021

Wnt/β-catenin pathway and cell adhesion deregulation in CSDE1-related intellectual disability and autism spectrum disorders.

E El Khouri, J Ghoumid, D Haye, F Giuliano, L Drevillon, A Briand-Suleau, P De La Grange, V Nau, T Gaillon, T Bienvenu and 4 more

Open access · greenAbstract readCase Reports
PubMed Publisher
In one paragraph

Article in Molecular psychiatry, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.

0numbers the graph read from it
0cells of the map it votes in
13citing papers in PubMed
1.1field-weighted citation impact, top 23% 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

13 citing papers in PubMed, 19 citations in OpenAlex.

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  12. CTNNB1 in neurodevelopmental disorders.Frontiers in psychiatry · 2023
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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

14 authors at 5 institutions in 1 country.

E El KhouriSorbonne Université, INSERM, Maladies génétiques d'expression pédiatrique, Département de Génétique médicale, Assistance Publique Hôpitaux de Paris, Hôpital Trousseau, Paris, France.ORCID http://orcid.org/0000-0002-4851-9632
J GhoumidDépartement de Génétique, Groupe Hospitalier Henri Mondor, Créteil, France.ORCID http://orcid.org/0000-0002-7111-0050
D HayeService de Génétique Médicale Centre, Hospitalo-Universitaire de Nice, Nice, France.
F GiulianoService de Génétique Médicale Centre, Hospitalo-Universitaire de Nice, Nice, France.
L DrevillonDépartement de Génétique, Groupe Hospitalier Henri Mondor, Créteil, France.
A Briand-SuleauDépartement de Génétique, Groupe Hospitalier Henri Mondor, Créteil, France.
P De La GrangeGenoSplice, Paris, France.
V NauSorbonne Université, INSERM, Maladies génétiques d'expression pédiatrique, Département de Génétique médicale, Assistance Publique Hôpitaux de Paris, Hôpital Trousseau, Paris, France.
T GaillonDépartement de Génétique, Groupe Hospitalier Henri Mondor, Créteil, France.
T BienvenuService de Génétique et Biologie Moléculaires, Hôpital Cochin, INSERM UMR1266 - Institute of Psychiatry and Neuroscience of Paris (IPNP) and University of Paris, Paris, France.
H Jacquemin-SablonINSERM UMR1053 Bordeaux Research in Translational Oncology, BaRITOn, Bordeaux, France.
M GoossensDépartement de Génétique, Groupe Hospitalier Henri Mondor, Créteil, France.
S AmselemSorbonne Université, INSERM, Maladies génétiques d'expression pédiatrique, Département de Génétique médicale, Assistance Publique Hôpitaux de Paris, Hôpital Trousseau, Paris, France.
I GiurgeaSorbonne Université, INSERM, Maladies génétiques d'expression pédiatrique, Département de Génétique médicale, Assistance Publique Hôpitaux de Paris, Hôpital Trousseau, Paris, France. irina.giurgea@inserm.fr.ORCID http://orcid.org/0000-0002-5035-2958
Inserm · FRCentre Hospitalier Universitaire Henri-Mondor · FRCentre Hospitalier Universitaire de Lille · FRCentre Hospitalier Universitaire de Nice · FRUniversité de Versailles Saint-Quentin-en-Yvelines · FR

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Among the genetic factors playing a key role in the etiology of intellectual disabilities (IDs) and autism spectrum disorders (ASDs), several encode RNA-binding proteins (RBPs). In this study, we deciphered the molecular and cellular bases of ID-ASD in a patient followed from birth to the age of 21, in whom we identified a de novo CSDE1 (Cold Shock Domain-containing E1) nonsense variation. CSDE1 encodes an RBP that regulates multiple cellular pathways by monitoring the translation and abundance of target transcripts. Analyses performed on the patient's primary fibroblasts showed that the identified CSDE1 variation leads to haploinsufficiency. We identified through RNA-seq assays the Wnt/β-catenin signaling and cellular adhesion as two major deregulated pathways. These results were further confirmed by functional studies involving Wnt-specific luciferase and substrate adhesion assays. Additional data support a disease model involving APC Down-Regulated-1 (APCDD1) and cadherin-2 (CDH2), two components of the Wnt/β-catenin pathway, CDH2 being also pivotal for cellular adhesion. Our study, which relies on both the deep phenotyping and long-term follow-up of a patient with CSDE1 haploinsufficiency and on ex vivo studies, sheds new light on the CSDE1-dependent deregulated pathways in ID-ASD.

Indexed as

Autism Spectrum DisorderDNA-Binding ProteinsIntellectual DisabilityRNA-Binding ProteinsWnt Signaling PathwayAdolescentbeta CateninCell AdhesionChildChild, PreschoolHumansInfantInfant, NewbornYoung Adultbeta CateninCSDE1 protein, humanDNA-Binding ProteinsRNA-Binding Proteins

Identifiers

PMID33867523
OpenAlexW3154406689

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.