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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Yang, H. Pullano, R. Shi, W. Filmus, J. Piscione, T. D. Soon, S. Mckerlie, C. Cano-gauci, D. F. Song, H. H. Choo, B. Rosenblum, N. D. Yeger, H. Mak, T. W. Lockwood, G. A. Grisaru, S. Sedlackova, L. Tanswell, A. K. |
| Description | Author Affiliation: Cano-Gauci DF ( The Ontario Cancer Institute, Toronto, Ontario, M5G 2M9 Canada.) |
| Abstract | Glypicans are a family of heparan sulfate proteoglycans that are linked to the cell surface through a glycosyl–phosphatidylinositol anchor. One member of this family, glypican-3 (Gpc3), is mutated in patients with the Simpson-Golabi-Behmel syndrome (SGBS). These patients display pre- and postnatal overgrowth, and a varying range of dysmorphisms. The clinical features of SGBS are very similar to the more extensively studied Beckwith-Wiedemann syndrome (BWS). Since BWS has been associated with biallelic expression of insulin-like growth factor II (IGF-II), it has been proposed that GPC3 is a negative regulator of IGF-II. However, there is still no biochemical evidence indicating that GPC3 plays such a role. Here, we report that GPC3-deficient mice exhibit several of the clinical features observed in SGBS patients, including developmental overgrowth, perinatal death, cystic and dyplastic kidneys, and abnormal lung development. A proportion of the mutant mice also display mandibular hypoplasia and an imperforate vagina. In the particular case of the kidney, we demonstrate that there is an early and persistent developmental abnormality of the ureteric bud/collecting system due to increased proliferation of cells in this tissue element. The degree of developmental overgrowth of the GPC3-deficient mice is similar to that of mice deficient in IGF receptor type 2 (IGF2R), a well characterized negative regulator of IGF-II. Unlike the IGF2R-deficient mice, however, the levels of IGF-II in GPC3 knockouts are similar to those of the normal littermates. |
| ISSN | 00219525 |
| e-ISSN | 15408140 |
| Journal | The Journal of Cell Biology |
| Issue Number | 1 |
| Volume Number | 146 |
| Language | English |
| Publisher | Rockefeller University Press (United States) |
| Publisher Date | 1999-07-12 |
| Publisher Place | United States |
| Access Restriction | Open |
| Subject Keyword | Abnormalities, Multiple Genetics Growth Disorders Heparan Sulfate Proteoglycans Heparitin Sulfate Deficiency Insulin-Like Growth Factor II Proteoglycans Physiopathology Animals Beckwith-Wiedemann Syndrome Body Weight Cell Division Genotype Glypicans Physiology Kidney Tubules, Collecting Abnormalities Embryology Pathology Mandible Mice Mice, Inbred C57BL Mice, Knockout Organ Size Phenotype Syndrome Research Support, Non-U.S. Gov't Cell Biology |
| Content Type | Text |
| Resource Type | Article |
| Subject | Cell Biology Medicine |
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