Microdeletion and IGF2 loss of imprinting in a cascade causing Beckwith-Wiedemann syndrome with Wilms' tumor
✍ Scribed by Prawitt, Dirk; Enklaar, Thorsten; Gärtner-Rupprecht, Barbara; Spangenberg, Christian; Lausch, Ekkehart; Reutzel, Dirk; Fees, Stephan; Korzon, Maria; Brozek, Izabela; Limon, Janusz
- Book ID
- 109916565
- Publisher
- Nature Publishing Group
- Year
- 2005
- Tongue
- English
- Weight
- 188 KB
- Volume
- 37
- Category
- Article
- ISSN
- 1061-4036
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## Abstract To evaluate the usefulness of regular radiographic screening to detect an asymptomatic intraabdominal tumor in patients with an increased risk of developing Wilms tumor, we reviewed the files of patients with hemihypertrophy, aniridia, or Beckwith‐Wiedemann syndrome who were registered
## Abstract The most common known molecular defect in Wilms tumor (WT) of the kidney, the most frequent solid tumor of childhood, is loss of imprinting (LOI) of the insulin‐like growth factor–II gene (__IGF2__), which involves activation of the normally silent maternal allele of the gene and hyperm