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Review

Molecular Biology of the WWOX Gene That Spans Chromosomal Fragile Site FRA16D

Department of Molecular and Biomedical Science, School of Biological Sciences, The University of Adelaide, Adelaide, SA 5000, Australia
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Academic Editor: Rami Aqeilan
Cells 2021, 10(7), 1637; https://doi.org/10.3390/cells10071637
Received: 9 April 2021 / Revised: 17 June 2021 / Accepted: 25 June 2021 / Published: 29 June 2021
(This article belongs to the Special Issue WWOX: A Fragile Tumor Suppressor with Pleotropic Functions)
It is now more than 20 years since the FRA16D common chromosomal fragile site was characterised and the WWOX gene spanning this site was identified. In this time, much information has been discovered about its contribution to disease; however, the normal biological role of WWOX is not yet clear. Experiments leading to the identification of the WWOX gene are recounted, revealing enigmatic relationships between the fragile site, its gene and the encoded protein. We also highlight research mainly using the genetically tractable model organism Drosophila melanogaster that has shed light on the integral role of WWOX in metabolism. In addition to this role, there are some particularly outstanding questions that remain regarding WWOX, its gene and its chromosomal location. This review, therefore, also aims to highlight two unanswered questions. Firstly, what is the biological relationship between the WWOX gene and the FRA16D common chromosomal fragile site that is located within one of its very large introns? Secondly, what is the actual substrate and product of the WWOX enzyme activity? It is likely that understanding the normal role of WWOX and its relationship to chromosomal fragility are necessary in order to understand how the perturbation of these normal roles results in disease. View Full-Text
Keywords: common chromosomal fragile sites; FRA16D; megabase gene; intragenic homozygous deletion; oxido-reductase specificity; evolutionary conservation common chromosomal fragile sites; FRA16D; megabase gene; intragenic homozygous deletion; oxido-reductase specificity; evolutionary conservation
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MDPI and ACS Style

Lee, C.S.; Choo, A.; Dayan, S.; Richards, R.I.; O’Keefe, L.V. Molecular Biology of the WWOX Gene That Spans Chromosomal Fragile Site FRA16D. Cells 2021, 10, 1637. https://doi.org/10.3390/cells10071637

AMA Style

Lee CS, Choo A, Dayan S, Richards RI, O’Keefe LV. Molecular Biology of the WWOX Gene That Spans Chromosomal Fragile Site FRA16D. Cells. 2021; 10(7):1637. https://doi.org/10.3390/cells10071637

Chicago/Turabian Style

Lee, Cheng S., Amanda Choo, Sonia Dayan, Robert I. Richards, and Louise V. O’Keefe 2021. "Molecular Biology of the WWOX Gene That Spans Chromosomal Fragile Site FRA16D" Cells 10, no. 7: 1637. https://doi.org/10.3390/cells10071637

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