Wwox-related neurodevelopmental disorders: Models and future perspectives

Daniel J. Steinberg; Rami I. Aqeilan

doi:https://doi.org/10.3390/cells10113082

Wwox-related neurodevelopmental disorders: Models and future perspectives

Daniel J. Steinberg, Rami I. Aqeilan

Department of Immunology and Cancer Research

The Hebrew University of Jerusalem

Research output: Contribution to journal › Review article › peer-review

Abstract

The WW domain-containing oxidoreductase (WWOX) 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.

Original language	English
Article number	3082
Journal	Cells
Volume	10
Issue number	11
DOIs	https://doi.org/10.3390/cells10113082
State	Published - 9 Nov 2021

Keywords

Brain organoids
Knockout
Models
SCAR12
WOREE syndrome

All Science Journal Classification (ASJC) codes

General Medicine

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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https://doi.org/10.3390/cells10113082

Cite this

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title = "Wwox-related neurodevelopmental disorders: Models and future perspectives",

abstract = "The WW domain-containing oxidoreductase (WWOX) 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.",

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AU - Aqeilan, Rami I.

N1 - Publisher Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https:// creativecommons.org/licenses/by/ 4.0/).

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AB - The WW domain-containing oxidoreductase (WWOX) 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.

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DO - https://doi.org/10.3390/cells10113082

M3 - مقالة مرجعية

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Wwox-related neurodevelopmental disorders: Models and future perspectives

Abstract

Keywords

All Science Journal Classification (ASJC) codes

UN SDGs

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