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...
| Main Authors: | , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2021-11-01
|
| Series: | Cells |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2073-4409/10/11/3082 |
| _version_ | 1827677050181255168 |
|---|---|
| author | Daniel J. Steinberg Rami I. Aqeilan |
| author_facet | Daniel J. Steinberg Rami I. Aqeilan |
| author_sort | Daniel J. Steinberg |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-10T05:36:45Z |
| format | Article |
| id | doaj.art-d1a4b639747b4d50b6f1ed132781d29b |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-03-10T05:36:45Z |
| publishDate | 2021-11-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cells |
| spelling | doaj.art-d1a4b639747b4d50b6f1ed132781d29b2023-11-22T22:51:02ZengMDPI AGCells2073-44092021-11-011011308210.3390/cells10113082WWOX-Related Neurodevelopmental Disorders: Models and Future PerspectivesDaniel J. Steinberg0Rami I. Aqeilan1The Concern Foundation Laboratories, The Lautenberg Center for Immunology and Cancer Research, Department of Immunology and Cancer Research-IMRIC, Faculty of Medicine, The Hebrew University of Jerusalem, Jerusalem 91120, IsraelThe Concern Foundation Laboratories, The Lautenberg Center for Immunology and Cancer Research, Department of Immunology and Cancer Research-IMRIC, Faculty of Medicine, The Hebrew University of Jerusalem, Jerusalem 91120, IsraelThe 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.https://www.mdpi.com/2073-4409/10/11/3082WOREE syndromeSCAR12brain organoidsknockoutmodels |
| spellingShingle | Daniel J. Steinberg Rami I. Aqeilan WWOX-Related Neurodevelopmental Disorders: Models and Future Perspectives Cells WOREE syndrome SCAR12 brain organoids knockout models |
| title | 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_short | WWOX-Related Neurodevelopmental Disorders: Models and Future Perspectives |
| title_sort | wwox related neurodevelopmental disorders models and future perspectives |
| topic | WOREE syndrome SCAR12 brain organoids knockout models |
| url | https://www.mdpi.com/2073-4409/10/11/3082 |
| work_keys_str_mv | AT danieljsteinberg wwoxrelatedneurodevelopmentaldisordersmodelsandfutureperspectives AT ramiiaqeilan wwoxrelatedneurodevelopmentaldisordersmodelsandfutureperspectives |