PMID: 11322381Apr 27, 2001Paper

Glypicans and the biology of renal malformations

Pediatric Nephrology : Journal of the International Pediatric Nephrology Association
S Grisaru, N D Rosenblum

Abstract

The molecular mechanisms that control renal development are largely undefined. The discovery of mutations in the gene encoding glypican-3 (Gpc3) in humans with Simpson-Golabi-Behmel syndrome (SGBS) and renal dysplasia, and the establishment of a genetic mouse model of GPC3 deficiency has provided an opportunity to define the role of GPC3 during renal development. Glypicans are a family of cell surface heparan sulfate proteoglycans that control growth factor signalling in nonrenal tissues. Mutational inactivation of Gpc3 causes somatic overgrowth and cystic renal dysplasia, as observed in SGBS. Overgrowth of the ureteric bud and its branches and increased ureteric bud cell proliferation is observed during the early stages of renal development. Subsequently, during corticomedullary differentiation, cortical collecting duct cell proliferation is increased, while medullary collecting duct cells proliferate at a reduced rate and undergo apoptosis resulting in degeneration of the medulla. However, cells that constitute medullary cysts are characterized by enhanced cell proliferation and a lower rate of apoptosis. Thus, the phenotype arising from Gpc3 inactivation demonstrates that tight regulation of cell proliferation and apoptosis ...Continue Reading

Citations

May 1, 2008·Nature Clinical Practice. Nephrology·Larissa KerecukAdrian S Woolf
Oct 9, 2003·Kidney International·Kristopher SchwabS Steven Potter
Feb 12, 2004·Kidney International·Angelique L W M M RopsJo H M Berden
Nov 5, 2014·Virchows Archiv : an International Journal of Pathology·Maria TretiakovaXiming J Yang
Jul 27, 2017·Clinical Dysmorphology·Takaya NakaneKanji Sugita
Jul 3, 2002·Nature Reviews. Genetics·Seppo Vainio, Yanfeng Lin
Sep 15, 2005·American Journal of Medical Genetics. Part a·Germán Rodríguez-CriadoPablo Lapunzina
Jan 7, 2004·American Journal of Physiology. Renal Physiology·Yashpal S KanwarJon W Lomasney

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