Pellegrini M, Pilia G, Pantano S, Lucchini F, Uda M, Fumi M, Cao A, Schlessinger D, Forabosco A
Dipartimento di Scienze Morfologiche e Medico Legali, Modena University, Italy.
Dev Dyn. 1998 Dec;213(4):431-9. doi: 10.1002/(SICI)1097-0177(199812)213:4<431::AID-AJA8>3.0.CO;2-7.
Interest in glypican-3 (GPC3), a member of the glypican-related integral membrane heparan sulfate proteoglycans (GRIPS) family, has increased with the finding that it is mutated in the Simpson-Golabi-Behmel overgrowth syndrome (Pilia et al. [1996] Nat. Genet. 12:241-247). The working model suggested that the membrane-bound protein acts locally to limit tissue and organ growth and that it may function by interacting with insulin-like growth factor 2 (IGF2) to limit its local effective level. Here we have tested two predictions of the model. In situ hybridization with the mouse gene cDNA was used to study the expression pattern during embryonic and fetal development. In agreement with predictions, the gene is expressed in precisely the organs that overgrow in its absence; and the patterns of expression of Gpc3 and those reported for Igf2 are strictly correlated.
对硫酸乙酰肝素蛋白聚糖相关整合膜蛋白聚糖（GRIPS）家族成员磷脂酰肌醇蛋白聚糖-3（GPC3）的关注随着以下发现而增加：它在辛普森-戈拉比-贝梅尔过度生长综合征中发生突变（皮利亚等人，[1996]《自然遗传学》12:241 - 247）。该工作模型表明，这种膜结合蛋白在局部发挥作用以限制组织和器官生长，并且它可能通过与胰岛素样生长因子2（IGF2）相互作用来限制其局部有效水平。在此，我们对该模型的两个预测进行了测试。利用小鼠基因cDNA进行原位杂交，以研究胚胎和胎儿发育过程中的表达模式。与预测一致，该基因恰好表达于在其缺失时会过度生长的器官中；并且Gpc3的表达模式与报道的Igf2的表达模式严格相关。
