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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Autores principales:	Daniel J. Steinberg, Rami I. Aqeilan
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	WOREE syndrome SCAR12 brain organoids knockout models Biology (General) QH301-705.5
Acceso en línea:	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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WWOX-Related Neurodevelopmental Disorders: Models and Future Perspectives

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