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Overgrowth syndromes and pediatric cancers: how many roads lead to IGF2?

Overgrowth syndromes such as Perlman syndrome and associated pediatric cancers, including Wilms tumor, arise through genetic and, in certain instances, also epigenetic changes. In the case of the Beckwith-Wiedemann overgrowth syndrome and in Wilms tumor, increased levels of IGF2 have been shown to b...

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Autores principales: Bharathavikru, Ruthrothaselvi, Hastie, Nicholas D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cold Spring Harbor Laboratory Press 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6075144/
https://www.ncbi.nlm.nih.gov/pubmed/30068702
http://dx.doi.org/10.1101/gad.317792.118
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author Bharathavikru, Ruthrothaselvi
Hastie, Nicholas D.
author_facet Bharathavikru, Ruthrothaselvi
Hastie, Nicholas D.
author_sort Bharathavikru, Ruthrothaselvi
collection PubMed
description Overgrowth syndromes such as Perlman syndrome and associated pediatric cancers, including Wilms tumor, arise through genetic and, in certain instances, also epigenetic changes. In the case of the Beckwith-Wiedemann overgrowth syndrome and in Wilms tumor, increased levels of IGF2 have been shown to be causally related to the disease manifestation. In the previous issue of Genes & Development, Hunter and colleagues (pp. 903–908) investigated the molecular mechanisms by which mutations in the gene encoding the RNA degradation component DIS3L2 lead to Perlman syndrome. By analyzing nephron progenitor cells derived from their newly created Dis3l2 mutant mouse lines, the investigators showed that DIS3L2 loss of function leads to up-regulation of IGF2 independently of the let7 microRNA pathway. In a second study in this issue of Genes & Development, Chen and colleagues (pp. 996–1007) show that microRNA processing gene mutations in Wilms tumor lead to an increase in the levels of transcription factor pleomorphic adenoma gene 1 (PLAG1) that in turn activates IGF2 expression. Thus, augmented IGF2 expression seems to be a common downstream factor in both tissue overgrowth and Wilms tumor through several alternative mechanisms.
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spelling pubmed-60751442018-08-17 Overgrowth syndromes and pediatric cancers: how many roads lead to IGF2? Bharathavikru, Ruthrothaselvi Hastie, Nicholas D. Genes Dev Perspective Overgrowth syndromes such as Perlman syndrome and associated pediatric cancers, including Wilms tumor, arise through genetic and, in certain instances, also epigenetic changes. In the case of the Beckwith-Wiedemann overgrowth syndrome and in Wilms tumor, increased levels of IGF2 have been shown to be causally related to the disease manifestation. In the previous issue of Genes & Development, Hunter and colleagues (pp. 903–908) investigated the molecular mechanisms by which mutations in the gene encoding the RNA degradation component DIS3L2 lead to Perlman syndrome. By analyzing nephron progenitor cells derived from their newly created Dis3l2 mutant mouse lines, the investigators showed that DIS3L2 loss of function leads to up-regulation of IGF2 independently of the let7 microRNA pathway. In a second study in this issue of Genes & Development, Chen and colleagues (pp. 996–1007) show that microRNA processing gene mutations in Wilms tumor lead to an increase in the levels of transcription factor pleomorphic adenoma gene 1 (PLAG1) that in turn activates IGF2 expression. Thus, augmented IGF2 expression seems to be a common downstream factor in both tissue overgrowth and Wilms tumor through several alternative mechanisms. Cold Spring Harbor Laboratory Press 2018-08-01 /pmc/articles/PMC6075144/ /pubmed/30068702 http://dx.doi.org/10.1101/gad.317792.118 Text en © 2018 Bharathavikru and Hastie; Published by Cold Spring Harbor Laboratory Press http://creativecommons.org/licenses/by/4.0/ This article, published in Genes & Development, is available under a Creative Commons License (Attribution 4.0 International), as described at http://creativecommons.org/licenses/by/4.0/.
spellingShingle Perspective
Bharathavikru, Ruthrothaselvi
Hastie, Nicholas D.
Overgrowth syndromes and pediatric cancers: how many roads lead to IGF2?
title Overgrowth syndromes and pediatric cancers: how many roads lead to IGF2?
title_full Overgrowth syndromes and pediatric cancers: how many roads lead to IGF2?
title_fullStr Overgrowth syndromes and pediatric cancers: how many roads lead to IGF2?
title_full_unstemmed Overgrowth syndromes and pediatric cancers: how many roads lead to IGF2?
title_short Overgrowth syndromes and pediatric cancers: how many roads lead to IGF2?
title_sort overgrowth syndromes and pediatric cancers: how many roads lead to igf2?
topic Perspective
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6075144/
https://www.ncbi.nlm.nih.gov/pubmed/30068702
http://dx.doi.org/10.1101/gad.317792.118
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