WWOX-Related Neurodevelopmental Disorders: Models and Future Perspectives

The WW domain-containing oxidoreductase (<i>WWOX</i>) gene was originally discovered as a putative tumor suppressor spanning the common fragile site FRA16D, but as time has progressed the extent of its pleiotropic function has become apparent. At present, WWOX is a major source of intere...

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Auteurs principaux: Daniel J. Steinberg, Rami I. Aqeilan
Format: article
Langue:EN
Publié: MDPI AG 2021
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Accès en ligne:https://doaj.org/article/d1a4b639747b4d50b6f1ed132781d29b
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spelling oai:doaj.org-article:d1a4b639747b4d50b6f1ed132781d29b2021-11-25T17:11:19ZWWOX-Related Neurodevelopmental Disorders: Models and Future Perspectives10.3390/cells101130822073-4409https://doaj.org/article/d1a4b639747b4d50b6f1ed132781d29b2021-11-01T00:00:00Zhttps://www.mdpi.com/2073-4409/10/11/3082https://doaj.org/toc/2073-4409The WW domain-containing oxidoreductase (<i>WWOX</i>) gene was originally discovered as a putative tumor suppressor spanning the common fragile site FRA16D, but as time has progressed the extent of its pleiotropic function has become apparent. At present, WWOX is a major source of interest in the context of neurological disorders, and more specifically developmental and epileptic encephalopathies (DEEs). This review article aims to introduce the many model systems used through the years to study its function and roles in neuropathies. Similarities and fundamental differences between rodent and human models are discussed. Finally, future perspectives and promising research avenues are suggested.Daniel J. SteinbergRami I. AqeilanMDPI AGarticleWOREE syndromeSCAR12brain organoidsknockoutmodelsBiology (General)QH301-705.5ENCells, Vol 10, Iss 3082, p 3082 (2021)
institution DOAJ
collection DOAJ
language EN
topic WOREE syndrome
SCAR12
brain organoids
knockout
models
Biology (General)
QH301-705.5
spellingShingle WOREE syndrome
SCAR12
brain organoids
knockout
models
Biology (General)
QH301-705.5
Daniel J. Steinberg
Rami I. Aqeilan
WWOX-Related Neurodevelopmental Disorders: Models and Future Perspectives
description The WW domain-containing oxidoreductase (<i>WWOX</i>) gene was originally discovered as a putative tumor suppressor spanning the common fragile site FRA16D, but as time has progressed the extent of its pleiotropic function has become apparent. At present, WWOX is a major source of interest in the context of neurological disorders, and more specifically developmental and epileptic encephalopathies (DEEs). This review article aims to introduce the many model systems used through the years to study its function and roles in neuropathies. Similarities and fundamental differences between rodent and human models are discussed. Finally, future perspectives and promising research avenues are suggested.
format article
author Daniel J. Steinberg
Rami I. Aqeilan
author_facet Daniel J. Steinberg
Rami I. Aqeilan
author_sort Daniel J. Steinberg
title WWOX-Related Neurodevelopmental Disorders: Models and Future Perspectives
title_short WWOX-Related Neurodevelopmental Disorders: Models and Future Perspectives
title_full WWOX-Related Neurodevelopmental Disorders: Models and Future Perspectives
title_fullStr WWOX-Related Neurodevelopmental Disorders: Models and Future Perspectives
title_full_unstemmed WWOX-Related Neurodevelopmental Disorders: Models and Future Perspectives
title_sort wwox-related neurodevelopmental disorders: models and future perspectives
publisher MDPI AG
publishDate 2021
url https://doaj.org/article/d1a4b639747b4d50b6f1ed132781d29b
work_keys_str_mv AT danieljsteinberg wwoxrelatedneurodevelopmentaldisordersmodelsandfutureperspectives
AT ramiiaqeilan wwoxrelatedneurodevelopmentaldisordersmodelsandfutureperspectives
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