@article{336de72283994be5b841b42be7e383dc,
title = "Loss of Dis3l2 partially phenocopies perlman syndrome in mice and results in upregulation of Igf2 in nephron progenitor cells",
abstract = "Loss of function of the DIS3L2 exoribonuclease is associated with Wilms tumor and the Perlman congenital overgrowth syndrome. LIN28, a Wilms tumor oncoprotein, triggers the DIS3L2-mediated degradation of the precursor of let-7, a microRNA that inhibits Wilms tumor development. These observations have led to speculation that DIS3L2-mediated tumor suppression is attributable to let-7 regulation. Here we examine new DIS3L2-deficient cell lines and mouse models, demonstrating that DIS3L2 loss has no effect on mature let-7 levels. Rather, analysis of Dis3l2-null nephron progenitor cells, a potential cell of origin of Wilms tumors, reveals up-regulation of Igf2, a growth-promoting gene strongly associated with Wilms tumorigenesis. These findings nominate a new potential mechanism underlying the pathology associated with DIS3L2 deficiency.",
keywords = "DIS3L2, Igf2, LIN28, Let-7, MicroRNA, Perlman syndrome, Wilms tumor",
author = "Hunter, {Ryan W.} and Yangjian Liu and Hema Manjunath and Asha Acharya and Jones, {Benjamin T.} and He Zhang and Beibei Chen and Harini Ramalingam and Hammer, {Robert E.} and Yang Xie and Richardson, {James A.} and Dinesh Rakheja and Carroll, {Thomas J.} and Mendell, {Joshua T.}",
note = "Funding Information: We thank Richard Gregory, Keith Joung, and Feng Zhang for plasmids and cell lines, and Vanessa Schmid in the McDermott Center Next-Generation Sequencing Core, John Shelton in the University of Texas South-western Histopathology Core, and Kathryn O{\textquoteright}Donnell and Kenneth Chen for helpful discussions. This work was supported by grants from the Cancer Prevention and Research Institute of Texas (RP160249 to J.T.M., D.R., and T.J.C., and RP150596 to the University of Texas South-western Bioinformatics Core Facility), the National Institutes of Health (R35CA197311 to J.T.M.; R24DK080004, R01DK095057, R01DK106743, and P30DK079328 to T.J.C.; and P50CA196516 to Y.X., D.R., T.J.C., and J.T.M.), and the Welch Foundation (I-1961-20180324 to J.T.M.). H.R. was supported by a University of Texas Southwestern Center for Regenerative Science and Medicine fellowship. J.T.M. is an Investigator of the Howard Hughes Medical Institute. Publisher Copyright: {\textcopyright} 2018 Hunter et al.",
year = "2018",
month = jul,
day = "1",
doi = "10.1101/gad.315804.118",
language = "English",
volume = "32",
pages = "903--908",
journal = "Genes and Development",
issn = "0890-9369",
number = "13-14",
}