Wilms' tumour: reconciling genetics and biology.
Wilms' tumour, a paediatric malignancy of the kidney, is a striking example of the relationship between aberrant development and cancer. Several different genetic loci have been implicated in the aetiology of the tumour; genomic imprinting also plays a role. One Wilms' tumour predisposition gene (WT1), encoding a zinc finger protein, is expressed in a limited set of tissues, including developing nephrons and gonads. The biology and genetics of Wilms' tumour underline the developmental relationship between kidneys and gonads.
Van Heyningen
V
V
MRC Human Genetics Unit, Western General Hospital, Edinburgh, UK.
Hastie
N D
ND
D000015:Abnormalities, Multiple / Q000235:genetics; D015783:Aniridia / Q000235:genetics; D002880:Chromosomes, Human, Pair 11; D003456:Cryptorchidism / Q000235:genetics; D004268:DNA-Binding Proteins / Q000502:physiology; D005260:Female; D018507:Gene Expression Regulation, Developmental; D015972:Gene Expression Regulation, Neoplastic; D016162:Genes, Wilms Tumor; D006066:Gonads / Q000196:embryology; D006801:Humans; D007021:Hypospadias / Q000235:genetics; D007668:Kidney / Q000196:embryology*; D007680:Kidney Neoplasms / Q000196:embryology / Q000235:genetics*; D008297:Male; D009857:Oncogenes; D017384:Sequence Deletion; D013577:Syndrome; D014157:Transcription Factors / Q000502:physiology; D025721:WT1 Proteins; D009396:Wilms Tumor / Q000196:embryology / Q000235:genetics*; D016335:Zinc Fingers
D004268:DNA-Binding Proteins; D014157:Transcription Factors; D025721:WT1 Proteins