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10.1007/s00467-013-2661-y http://scihub22266oqcxt.onion/10.1007/s00467-013-2661-y C3951435!3951435!24271660     free     free     free Deprecated: Implicit conversion from float 219.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 219.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 219.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 219.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 219.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       Pediatr+Nephrol 2014 ; 29 (4): 651-7 Nephropedia Template TP {{tp|p=24271660|t=2014. Alagille, Notch, and robustness: why duplicating systems does not produce redundancy |pdf=|usr=}}{{24271660}} gab.com Text Alagille, Notch, and robustness: why duplicating systems does not produce redundancy JO: Pediatr Nephrol http://www.kidney.de/pget.php?&tw=1&pmid=24271660 #FOAvip The mammalian kidney forms from several populations of progenitors that only persist during embryogenesis. The epithelial nephron progenitors reside in the cap mesenchyme (CM), whereas mesangial and endothelial cell progenitors reside in the neighboring stromal mesenchyme (SM). After a ureteric bud (UB) signal induces mesenchymal to epithelial transition of some CM cells, they form a nascent epithelial ball (a renal vesicle or RV) that require signals mediated by Notch receptors to separate proximal from distal fates. Two Notch receptors (Notch1 and Notch2) and two ligands (Jagged1 and Delta1) are expressed in the RV. Notably, instead of providing sufficient redundancy to ensure that losing any one allele will be inconsequential to human health, a reduction in the dose of one ligand (Jagged1) or one receptor (Notch2) is causally associated with a rare developmental syndrome (Alagille syndrome, ALGS) affecting eye, kidney, liver, and cranio-facial development. Here we will discuss our current understanding of the molecular basis for the non-redundant role of Notch2 in this process, and the avenue for new therapeutic strategies that these insights provide. Twit Text FOAVip Alagille, Notch, and robustness: why duplicating systems does not produce redundancy ????Pediatr Nephrol http://www.kidney.de/pget.php?&tw=1&pmid=24271660 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=24271660 #FOAvip English Wikipedia <ref>{{Cite pmid|24271660}}</ref> Alagille, Notch, and robustness: why duplicating systems does not produce redundancy #MMPMID24271660 Kopan R; Chen S; Liu Z Pediatr Nephrol 2014[Apr]; 29 (4): 651-7 PMID24271660show ga The mammalian kidney forms from several populations of progenitors that only persist during embryogenesis. The epithelial nephron progenitors reside in the cap mesenchyme (CM), whereas mesangial and endothelial cell progenitors reside in the neighboring stromal mesenchyme (SM). After a ureteric bud (UB) signal induces mesenchymal to epithelial transition of some CM cells, they form a nascent epithelial ball (a renal vesicle or RV) that require signals mediated by Notch receptors to separate proximal from distal fates. Two Notch receptors (Notch1 and Notch2) and two ligands (Jagged1 and Delta1) are expressed in the RV. Notably, instead of providing sufficient redundancy to ensure that losing any one allele will be inconsequential to human health, a reduction in the dose of one ligand (Jagged1) or one receptor (Notch2) is causally associated with a rare developmental syndrome (Alagille syndrome, ALGS) affecting eye, kidney, liver, and cranio-facial development. Here we will discuss our current understanding of the molecular basis for the non-redundant role of Notch2 in this process, and the avenue for new therapeutic strategies that these insights provide. äAlagille, Notch, and robustness: why duplicating systems does not prod(epmc).pdf DeepDyve Pubget Overpricing