Wilms' tumors and malformation complexes

J. Hata, R. Fukuzawa, A. Takata, H. Kikuchi

Research output: Contribution to journalArticle

Abstract

Wilms' tumor is an embryonal tumor which is derived from metanephric metanephric blastema. The occurrence of both sporadic and hereditary forms, along with various congenital abnormalities of Wilms' tumor suggest that the tumors develop when a predisposing germ line mutation is accompanied by a second mutation. The existence of both gross chromosomal abnormalities has led to the genetic characterization of a number of loci involved in the development of Wilms' tumor. A tumor suppressor gene for Wilms' tumor, WT1, has been isolated from the 11p13 region. The product of this gene is a transcription factor with four zinc fingers. Because of expression of WT1 is limited to the developing glomeruli of the kidneys and the genital ridge, it is thought to have a functional role in renal and gonadal organogesis. Thus dysfunction of WT1 causes loss of normal regulation of proliferation and leads to tumor formation and occurrence of Wilms' tumor anomaly complexes. The role of the imprinting genes, H19 and IGF2 in oncogenesis of Wilms' tumors are also discussed.

Original languageEnglish
Pages (from-to)1419-1425
Number of pages7
JournalNippon rinsho. Japanese journal of clinical medicine
Volume58
Issue number7
Publication statusPublished - 2000

Fingerprint

Wilms Tumor
Kidney Glomerulus
Neoplasms
Germ-Line Mutation
Zinc Fingers
Tumor Suppressor Genes
Chromosome Aberrations
Genes
Carcinogenesis
Transcription Factors
Kidney
Mutation

Cite this

Wilms' tumors and malformation complexes. / Hata, J.; Fukuzawa, R.; Takata, A.; Kikuchi, H.

In: Nippon rinsho. Japanese journal of clinical medicine, Vol. 58, No. 7, 2000, p. 1419-1425.

Research output: Contribution to journalArticle

Hata, J, Fukuzawa, R, Takata, A & Kikuchi, H 2000, 'Wilms' tumors and malformation complexes', Nippon rinsho. Japanese journal of clinical medicine, vol. 58, no. 7, pp. 1419-1425.
Hata, J. ; Fukuzawa, R. ; Takata, A. ; Kikuchi, H. / Wilms' tumors and malformation complexes. In: Nippon rinsho. Japanese journal of clinical medicine. 2000 ; Vol. 58, No. 7. pp. 1419-1425.
@article{8adb490a35524b649dcab5cd7b481a87,
title = "Wilms' tumors and malformation complexes",
abstract = "Wilms' tumor is an embryonal tumor which is derived from metanephric metanephric blastema. The occurrence of both sporadic and hereditary forms, along with various congenital abnormalities of Wilms' tumor suggest that the tumors develop when a predisposing germ line mutation is accompanied by a second mutation. The existence of both gross chromosomal abnormalities has led to the genetic characterization of a number of loci involved in the development of Wilms' tumor. A tumor suppressor gene for Wilms' tumor, WT1, has been isolated from the 11p13 region. The product of this gene is a transcription factor with four zinc fingers. Because of expression of WT1 is limited to the developing glomeruli of the kidneys and the genital ridge, it is thought to have a functional role in renal and gonadal organogesis. Thus dysfunction of WT1 causes loss of normal regulation of proliferation and leads to tumor formation and occurrence of Wilms' tumor anomaly complexes. The role of the imprinting genes, H19 and IGF2 in oncogenesis of Wilms' tumors are also discussed.",
author = "J. Hata and R. Fukuzawa and A. Takata and H. Kikuchi",
year = "2000",
language = "English",
volume = "58",
pages = "1419--1425",
journal = "Nihon rinsho. Japanese journal of clinical medicine",
issn = "0047-1852",
publisher = "Nipponrinsho Co., Inc.",
number = "7",

}

TY - JOUR

T1 - Wilms' tumors and malformation complexes

AU - Hata, J.

AU - Fukuzawa, R.

AU - Takata, A.

AU - Kikuchi, H.

PY - 2000

Y1 - 2000

N2 - Wilms' tumor is an embryonal tumor which is derived from metanephric metanephric blastema. The occurrence of both sporadic and hereditary forms, along with various congenital abnormalities of Wilms' tumor suggest that the tumors develop when a predisposing germ line mutation is accompanied by a second mutation. The existence of both gross chromosomal abnormalities has led to the genetic characterization of a number of loci involved in the development of Wilms' tumor. A tumor suppressor gene for Wilms' tumor, WT1, has been isolated from the 11p13 region. The product of this gene is a transcription factor with four zinc fingers. Because of expression of WT1 is limited to the developing glomeruli of the kidneys and the genital ridge, it is thought to have a functional role in renal and gonadal organogesis. Thus dysfunction of WT1 causes loss of normal regulation of proliferation and leads to tumor formation and occurrence of Wilms' tumor anomaly complexes. The role of the imprinting genes, H19 and IGF2 in oncogenesis of Wilms' tumors are also discussed.

AB - Wilms' tumor is an embryonal tumor which is derived from metanephric metanephric blastema. The occurrence of both sporadic and hereditary forms, along with various congenital abnormalities of Wilms' tumor suggest that the tumors develop when a predisposing germ line mutation is accompanied by a second mutation. The existence of both gross chromosomal abnormalities has led to the genetic characterization of a number of loci involved in the development of Wilms' tumor. A tumor suppressor gene for Wilms' tumor, WT1, has been isolated from the 11p13 region. The product of this gene is a transcription factor with four zinc fingers. Because of expression of WT1 is limited to the developing glomeruli of the kidneys and the genital ridge, it is thought to have a functional role in renal and gonadal organogesis. Thus dysfunction of WT1 causes loss of normal regulation of proliferation and leads to tumor formation and occurrence of Wilms' tumor anomaly complexes. The role of the imprinting genes, H19 and IGF2 in oncogenesis of Wilms' tumors are also discussed.

UR - http://www.scopus.com/inward/record.url?scp=0034222257&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0034222257&partnerID=8YFLogxK

M3 - Article

C2 - 10921316

AN - SCOPUS:0034222257

VL - 58

SP - 1419

EP - 1425

JO - Nihon rinsho. Japanese journal of clinical medicine

JF - Nihon rinsho. Japanese journal of clinical medicine

SN - 0047-1852

IS - 7

ER -