Decreased nuclear Pten in neural stem cells contributes to deficits in neuronal maturation.


Journal

Molecular autism
ISSN: 2040-2392
Titre abrégé: Mol Autism
Pays: England
ID NLM: 101534222

Informations de publication

Date de publication:
01 06 2020
Historique:
received: 30 09 2019
accepted: 13 04 2020
entrez: 4 6 2020
pubmed: 4 6 2020
medline: 29 5 2021
Statut: epublish

Résumé

PTEN, a syndromic autism spectrum disorder (ASD) risk gene, is mutated in approximately 10% of macrocephalic ASD cases. Despite the described genetic association between PTEN and ASD and ensuing studies, we continue to have a limited understanding of how PTEN disruption drives ASD pathogenesis and maintenance. We derived neural stem cells (NSCs) from the dentate gyrus (DG) of Pten The m3m4 mutation decreases Pten localization to the nucleus and its global expression over time. Pten Constitutional disruption of Pten nuclear localization with subsequent global decrease in Pten expression generates abnormal patterns of differentiation, a stunting of neuronal maturation. The propensity of Pten disruption to restrain neurons to a more progenitor-like state may be an important feature contributing to PTEN-ASD pathogenesis.

Sections du résumé

BACKGROUND
PTEN, a syndromic autism spectrum disorder (ASD) risk gene, is mutated in approximately 10% of macrocephalic ASD cases. Despite the described genetic association between PTEN and ASD and ensuing studies, we continue to have a limited understanding of how PTEN disruption drives ASD pathogenesis and maintenance.
METHODS
We derived neural stem cells (NSCs) from the dentate gyrus (DG) of Pten
RESULTS
The m3m4 mutation decreases Pten localization to the nucleus and its global expression over time. Pten
CONCLUSIONS
Constitutional disruption of Pten nuclear localization with subsequent global decrease in Pten expression generates abnormal patterns of differentiation, a stunting of neuronal maturation. The propensity of Pten disruption to restrain neurons to a more progenitor-like state may be an important feature contributing to PTEN-ASD pathogenesis.

Identifiants

pubmed: 32487265
doi: 10.1186/s13229-020-00337-2
pii: 10.1186/s13229-020-00337-2
pmc: PMC7268763
doi:

Substances chimiques

Biomarkers 0
PTEN Phosphohydrolase EC 3.1.3.67
PTEN protein, human EC 3.1.3.67

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

43

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Auteurs

Shin Chung Kang (SC)

Genomic Medicine Institute, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, 44195, USA.

Ritika Jaini (R)

Genomic Medicine Institute, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, 44195, USA.
Cleveland Clinic Lerner College of Medicine, Case Western Reserve University, 9500 Euclid Avenue, Cleveland, OH, 44195, USA.
Case Comprehensive Cancer Center, Case Western Reserve University School of Medicine, Cleveland, OH, 44106, USA.

Masahiro Hitomi (M)

Genomic Medicine Institute, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, 44195, USA.
Cleveland Clinic Lerner College of Medicine, Case Western Reserve University, 9500 Euclid Avenue, Cleveland, OH, 44195, USA.

Hyunpil Lee (H)

Genomic Medicine Institute, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, 44195, USA.

Nick Sarn (N)

Genomic Medicine Institute, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, 44195, USA.
Department of Genetics and Genome Sciences, Case Western Reserve University School of Medicine, Cleveland, OH, 44106, USA.

Stetson Thacker (S)

Genomic Medicine Institute, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, 44195, USA.
Cleveland Clinic Lerner College of Medicine, Case Western Reserve University, 9500 Euclid Avenue, Cleveland, OH, 44195, USA.

Charis Eng (C)

Genomic Medicine Institute, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, 44195, USA. engc@ccf.org.
Cleveland Clinic Lerner College of Medicine, Case Western Reserve University, 9500 Euclid Avenue, Cleveland, OH, 44195, USA. engc@ccf.org.
Case Comprehensive Cancer Center, Case Western Reserve University School of Medicine, Cleveland, OH, 44106, USA. engc@ccf.org.
Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, 44195, USA. engc@ccf.org.
Department of Genetics and Genome Sciences, Case Western Reserve University School of Medicine, Cleveland, OH, 44106, USA. engc@ccf.org.

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