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| Somatic overgrowth associated with overexpression of insulin-like growth factor II |
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| Author(s): Morison IM, Becroft DM, Taniguchi T, Woods CG, Reeve AE |
| Source: NATURE MEDICINE Volume: 2 Issue: 3 Pages: 311-316 Published: MAR 1996 |
| Times Cited: 94 References: 52 |
| Abstract: Overexpression of the normally imprinted fetal insulin-like growth factor II (IGF2) has been implicated in the pathogenesis of the cancer-predisposing Beckwith-Wiedemann syndrome (BWS). We have detected constitutional relaxation of imprinting of IGF2 in four children with somatic overgrowth who do not show diagnostic features of BWS. Three children showed constitutional abnormalities of H19 methylation. All four children showed nephromegaly and two developed Wilms' tumors. Gene methylation is known to be associated with gene silencing, and three children showed constitutional abnormalities of H19 gene methylation. Disruption of H19 methylation, and concomitant relaxation of IGF2 imprinting, provides another mechanism that can increase IGF2 expression in children with overgrowth. The accumulated data on normal and pathologic IGF2 expression are now sufficient to define an entity, ''IGF2 overgrowth disorder,'' of which BWS may be one extreme manifestation. These findings have broad implications for the characterization of idiopathic overgrowth. |
| Document Type: Article |
| Language: English |
Addresses:
1. UNIV OTAGO, DEPT BIOCHEM, CANC GENET LAB, DUNEDIN, NEW ZEALAND
2. UNIV AUCKLAND, DEPT OBSTET & GYNAECOL, AUCKLAND, NEW ZEALAND
3. ST JAMESS UNIV HOSP, YORKSHIRE REG GENET SERV, LEEDS, W YORKSHIRE ENGLAND |
| Publisher: NATURE PUBLISHING CO, 345 PARK AVE SOUTH, NEW YORK, NY 10010-1707 |
| Subject Category: Biochemistry & Molecular Biology; Cell Biology; Medicine, Research & Experimental |
| IDS Number: TY640 |
| ISSN: 1078-8956 |
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