Hedborg F, Holmgren L, Sandstedt B, Ohlsson R
Department of Drug Dependence Research, Karolinska Hospital, Stockholm, Sweden.
Am J Pathol. 1994 Oct;145(4):802-17.
Overstimulation by insulin-like growth factor II is implied in several overgrowth conditions and childhood cancers. We have therefore studied spatial and temporal expression patterns of the insulin-like growth factor II gene (IGF2) and the insulin-like growth factor type 1 receptor gene during normal human development (5.5 to 23.0 weeks postfertilization). The set of cell types with the most abundant IGF2 expression correlated strikingly to the organomegaly and tumor predisposition of the Beckwith-Wiedemann syndrome. Intrauterine growth and postnatal organ weights of a prematurely born child with a full-blown syndrome are presented. The cell type-specific IGF2 expression of these organs and of multifocal Wilms' tumors from two other children affected by the Beckwith-Wiedemann syndrome were also studied. The results clarify and extend previous findings concerning human prenatal IGF2 expression and are consistent with a short range overstimulatory role of locally produced IGF II ensuing after the first trimester in the Beckwith-Wiedemann syndrome.
胰岛素样生长因子II的过度刺激与多种过度生长病症及儿童癌症相关。因此，我们研究了胰岛素样生长因子II基因（IGF2）和胰岛素样生长因子1型受体基因在人类正常发育过程中（受精后5.5至23.0周）的时空表达模式。IGF2表达最丰富的细胞类型与贝克威思-维德曼综合征的器官肿大和肿瘤易感性显著相关。本文展示了一名患有典型综合征的早产儿的宫内生长情况及出生后器官重量。我们还研究了这些器官以及另外两名受贝克威思-维德曼综合征影响儿童的多灶性威尔姆斯瘤的细胞类型特异性IGF2表达。这些结果阐明并扩展了先前关于人类产前IGF2表达的研究发现，且与贝克威思-维德曼综合征妊娠中期后局部产生的IGF II的短程过度刺激作用相一致。
