| First Author | Hunter RW | Year | 2018 |
| Journal | Genes Dev | Volume | 32 |
| Issue | 13-14 | Pages | 903-908 |
| PubMed ID | 29950491 | Mgi Jnum | J:272435 |
| Mgi Id | MGI:6284242 | Doi | 10.1101/gad.315804.118 |
| Citation | Hunter RW, et al. (2018) Loss of Dis3l2 partially phenocopies Perlman syndrome in mice and results in up-regulation of Igf2 in nephron progenitor cells. Genes Dev 32(13-14):903-908 |
| abstractText | 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. |
