Loss of heterozygosity in non-tumoral tissue in two children with Beckwith-Wiedemann syndrome

H Schneid; M P Vazquez; D Seurin; Y le Bouc

Loss of heterozygosity in non-tumoral tissue in two children with Beckwith-Wiedemann syndrome

Growth Regul. 1991 Dec;1(4):168-70.

Authors

H Schneid¹, M P Vazquez, D Seurin, Y le Bouc

Affiliation

¹ Institut National de la Santé et de la Recherche Médicale U.142, Hôpital Saint Antoine, Paris, France.

PMID: 1688177

Abstract

The Insulin-like growth factor II (IGF-II) gene has been analysed by Southern blotting in healthy and tumor tissue in two children with Beckwith-Wiedemann syndrome, using the enzymes, AvaII and SacI. Loss of heterozygosity was determined not only in the DNA of a nephroblastoma, but also in the healthy kidney, leukocytes and fragments of tongue removed for obstructive macroglossia. Allele loss therefore seems to be sufficient to provoke tumorigenesis in some tissues, but not in others.

Publication types

Case Reports
Research Support, Non-U.S. Gov't

MeSH terms

Beckwith-Wiedemann Syndrome / genetics*
Blotting, Southern
DNA, Neoplasm / analysis
Deoxyribonucleases, Type II Site-Specific
Female
Heterozygote*
Humans
Infant
Insulin-Like Growth Factor II / genetics*
Kidney Neoplasms / genetics
Leukocytes / chemistry
Male
Polymorphism, Restriction Fragment Length
Tongue / chemistry
Wilms Tumor / genetics

Substances

DNA, Neoplasm
Insulin-Like Growth Factor II
endodeoxyribonuclease SacI
Deoxyribonucleases, Type II Site-Specific
GGWCC-specific type II deoxyribonucleases