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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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) |
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WOREE syndrome SCAR12 brain organoids knockout models Biology (General) QH301-705.5 |
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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 |
_version_ |
1718412680482521088 |