Wilms tumour: a developmental anomaly.
Wilms tumour (WT) is a developmental anomaly of the kidney which results from loss of function of at least one so called tumour suppressor gene on chromosome 11. The position of the gene at chromosome 11p13 is known through the association of WT with aniridia (lack of an iris), mental retardation and genitourinary abnormalities in the WAGR syndrome. Here we discuss the high resolution mapping studies to locate the position of the gene and conclude that the gonadal abnormalities in WAGR patients may be due to a defect in the WT gene itself. In support of this role in genitourinary development we show that a candidate WT gene is expressed in specific regions of the developing kidney and in fetal and embryonic gonads.
Hastie
N D
ND
MRC Human Genetics Unit, Western General Hospital, Edinburgh, U.K.
Bickmore
W
W
Pritchard-Jones
K
K
Porteous
D J
DJ
van Heyningen
V
V
D000015:Abnormalities, Multiple / Q000235:genetics*; D000818:Animals; D015783:Aniridia / Q000196:embryology / Q000235:genetics*; D002675:Child, Preschool; D002869:Chromosome Aberrations / Q000235:genetics; D025063:Chromosome Disorders; D002874:Chromosome Mapping; D002880:Chromosomes, Human, Pair 11; D005123:Eye / Q000196:embryology; D005260:Female; D016161:Genes, Retinoblastoma; D016162:Genes, Wilms Tumor; D005819:Genetic Markers; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000235:genetics / Q000378:metabolism; D007680:Kidney Neoplasms / Q000196:embryology / Q000235:genetics*; D008297:Male; D051379:Mice; D012334:RNA, Neoplasm / Q000235:genetics; D013577:Syndrome; D014157:Transcription Factors / Q000235:genetics; D014564:Urogenital Abnormalities; D014566:Urogenital System / Q000196:embryology; D009396:Wilms Tumor / Q000196:embryology / Q000235:genetics*; D016335:Zinc Fingers / Q000235:genetics
D005819:Genetic Markers; D012334:RNA, Neoplasm; D014157:Transcription Factors