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Molecular Biology Intelligence Unit

Wilms Tumor: Clinical and Molecular Characterization

Authors: Coppes, Max J., Campbell, Christine E., Williams, Bryan

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WT1 is a DNA Binding Protein Containing Four C2H2 Zinc Fingers . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89 The DNA Binding Domain ofWT1 ··············································~··· 90 WT1 Inhibits Tag and SV40 Origin Dependent Replication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97 WT1 is an Unusual C2H2 Zinc Finger Protein . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 98 WT1 is a Transcriptional Regulator . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99 Possible Downstream Targets ofWT1 Transcriptional Regulation . . . 103 Protein-Protein Interactions Involving WT1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 104 Other Factors that May Contribute to or Modify the Cellular Function ofWT1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 104 WT1 and Apoptosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 106 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 107 7. Naturally Occurring Mutations in the WTJ Gene . . . . . . . . . . . . . . . . . . 113 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 113 WTI Mutations in Wilms Tumor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 114 Analysis of WTI Mutations in Tumors Other than Wilms Tumor . . . 122 Constitutional WTI Mutations and the Denys-Drash Syndrome . . . . . . 125 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 131 8. Other Loci Implicated in Wtlms Tumor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 137 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 137 Nephrogenic Rests, WTI Mutations and Wilms Tumor . . . . . . . . . . . . . . . . . . . 137 Undetectable WTI Mutations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 138 Wilms Tumor and WITI . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 140 Loss of Heterozygosity for Chromosome 1 p and 16q . . . . . . . . . . . . . . . . . . . . . . . . 141 Wilms Tumor and Beckwith-Wiedemann Syndrome . . . . . . . . . . . . . . . . . . . . . . . . 141 Wilms Tumor and Perlman Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 144 Imprinting, IGF/l, HI9 and Wilms Tumor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 144 Wilms Tumor and Li-F raumeni Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 149 Familial Wilms Tumor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 149 Wilms Tumor and Other Disease Associations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 150 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 150 Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15 7 =====PREFACE===== ilms tumor is a common pediatric neoplasm of the kidney which has been W considered a paradigm for understanding the etiology of embryonal tu­ mors.

Table of contents (8 chapters)

Table of contents (8 chapters)

Buy this book

eBook $84.99
price for USA in USD (gross)
  • ISBN 978-3-662-22621-6
  • Digitally watermarked, DRM-free
  • Included format: EPUB, PDF
  • ebooks can be used on all reading devices
  • Immediate eBook download after purchase
Softcover $109.00
price for USA in USD
  • ISBN 978-3-662-22623-0
  • Free shipping for individuals worldwide
  • Usually dispatched within 3 to 5 business days.
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Bibliographic Information

Bibliographic Information
Book Title
Wilms Tumor: Clinical and Molecular Characterization
Authors
Series Title
Molecular Biology Intelligence Unit
Copyright
1995
Publisher
Springer-Verlag Berlin Heidelberg
Copyright Holder
Springer-Verlag Berlin Heidelberg
Distribution Rights
Distribution rights for the USA, Canada and Mexico: R.G. Landes, Biomedical Publishers
eBook ISBN
978-3-662-22621-6
DOI
10.1007/978-3-662-22621-6
Softcover ISBN
978-3-662-22623-0
Series ISSN
1431-0414
Edition Number
1
Number of Pages
VII, 163
Number of Illustrations
24 b/w illustrations, 6 illustrations in colour
Additional Information
Jointly published with R.G. Landes, Biomedical Publishers, Austin, USA
Topics