Isoform-Specific Reduction of the Basic Helix-Loop-Helix Transcription Factor TCF4 Levels in Huntington's Disease.

Adult Animals Disease Models, Animal Hippocampus Humans Huntington Disease / genetics Male Mice Mice, Transgenic Neurons Protein Isoforms Transcription Factor 4 / genetics
Huntington’s disease TCF4 basic helix-loop-helix transcription factor neurodegenerative disease transcriptional regulation

Journal

eNeuro
ISSN: 2373-2822
Titre abrégé: eNeuro
Pays: United States
ID NLM: 101647362

Informations de publication

Date de publication:
Historique:
received: 03 05 2021
revised: 19 08 2021
accepted: 22 08 2021
pubmed: 15 9 2021
medline: 21 10 2021
entrez: 14 9 2021
Statut: epublish

Résumé

Huntington's disease (HD) is an inherited neurodegenerative disorder with onset of characteristic motor symptoms at midlife, preceded by subtle cognitive and behavioral disturbances. Transcriptional dysregulation emerges early in the disease course and is considered central to HD pathogenesis. Using wild-type (wt) and HD knock-in mouse striatal cell lines we observed a HD genotype-dependent reduction in the protein levels of transcription factor 4 (TCF4), a member of the basic helix-loop-helix (bHLH) family with critical roles in brain development and function. We characterized mouse

Identifiants

DOI: 10.1523/ENEURO.0197-21.2021 PMID: 34518368 PMC: PMC8519306
pubmed: 34518368
pii: ENEURO.0197-21.2021
doi: 10.1523/ENEURO.0197-21.2021
pmc: PMC8519306
pii:
doi:

Substances chimiques

Protein Isoforms 0
TCF4 protein, human 0
Transcription Factor 4 0

Types de publication

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

Langues

eng

Sous-ensembles de citation

IM

Subventions

Organisme : Medical Research Council
ID : MR/L010305/1
Pays : United Kingdom
Organisme : Medical Research Council
ID : MR/L010305/1
Pays : United Kingdom

Informations de copyright

Copyright © 2021 Nurm et al.

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Auteurs

Kaja Nurm (K)

Department of Chemistry and Biotechnology, Tallinn University of Technology, Tallinn 12618, Estonia.

Mari Sepp (M)

Department of Chemistry and Biotechnology, Tallinn University of Technology, Tallinn 12618, Estonia.
ORCID: 0000-0003-1733-8385

Carla Castany-Pladevall (C)

Departament de Biomedicina, Facultat de Medicina i Ciències de la Salut, Institut de Neurociències, Universitat de Barcelona, Barcelona, Catalonia 08036, Spain.
Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia 08036, Spain.
Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Madrid, 28031, Spain.

Jordi Creus-Muncunill (J)

Departament de Biomedicina, Facultat de Medicina i Ciències de la Salut, Institut de Neurociències, Universitat de Barcelona, Barcelona, Catalonia 08036, Spain.
Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia 08036, Spain.
Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Madrid, 28031, Spain.

Jürgen Tuvikene (J)

Department of Chemistry and Biotechnology, Tallinn University of Technology, Tallinn 12618, Estonia.
Protobios LLC, Tallinn 12618, Estonia.
ORCID: 0000-0002-9665-760X

Alex Sirp (A)

Department of Chemistry and Biotechnology, Tallinn University of Technology, Tallinn 12618, Estonia.

Hanna Vihma (H)

Department of Chemistry and Biotechnology, Tallinn University of Technology, Tallinn 12618, Estonia.

Derek J Blake (DJ)

Division of Psychological Medicine and Clinical Neurosciences, MRC Centre for Neuropsychiatric Genetics and Genomics, Cardiff University, Cardiff CF24 4HQ, United Kingdom.
ORCID: 0000-0002-5005-4731

Esther Perez-Navarro (E)

Departament de Biomedicina, Facultat de Medicina i Ciències de la Salut, Institut de Neurociències, Universitat de Barcelona, Barcelona, Catalonia 08036, Spain.
Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia 08036, Spain.
Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Madrid, 28031, Spain.

Tõnis Timmusk (T)

Department of Chemistry and Biotechnology, Tallinn University of Technology, Tallinn 12618, Estonia tonis.timmusk@taltech.ee.
Protobios LLC, Tallinn 12618, Estonia.

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Classifications MeSH

questionsmedicales.fr › Personnes › Tranches d'âge › Adulte › Isoform-Specific Reduction of the Basic...
questionsmedicales.fr › Eucaryotes › Animaux › Isoform-Specific Reduction of the Basic...
questionsmedicales.fr › Techniques d'investigation › Modèles théoriques › Modèles biologiques › Modèles animaux de maladie humaine › Isoform-Specific Reduction of the Basic...
questionsmedicales.fr › Système nerveux › Système nerveux central › Encéphale › Prosencéphale › Télencéphale › Cerveau › Cortex cérébral › Hippocampe › Isoform-Specific Reduction of the Basic...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Isoform-Specific Reduction of the Basic...
questionsmedicales.fr › Troubles mentaux › Troubles neurocognitifs › Démence › Maladie de Huntington › Isoform-Specific Reduction of the Basic...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Rodentia › Muridae › Murinae › Souris › Isoform-Specific Reduction of the Basic...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Rodentia › Muridae › Murinae › Souris › Souris transgéniques › Isoform-Specific Reduction of the Basic...
questionsmedicales.fr › Cellules › Neurones › Isoform-Specific Reduction of the Basic...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Isoformes de protéines › Isoform-Specific Reduction of the Basic...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Facteurs de transcription › Facteurs de transcription à motif basique hélice-boucle-hélice › Facteur-4 de transcription › Isoform-Specific Reduction of the Basic...