[Genetic alterations associated with pathologic differentiation of Wilms' tumors].
Although several cytogenetic alterations have been associated with development of Wilms' tumor, a multigenic neoplasia, molecular mechanisms of its induction, development and maintenance remain to be elucided. In order to characterize these different steps we have developed a unique animal model of Wilms' tumor constituted by the MAV-1(N) induced avian nephroblastoma. This animal model led to the discovery in our laboratory of a new gene now (nephroblastoma overexpressed gene) which is overexpressed in all avian nephroblastoma. Expression of the human nov gene (novH), which is down-regulated by WT1, is also deregulated in Wilms' tumors. Nov characteristics suggest that it would play a role in the control of cellular proliferation and differentiation. Our observations also indicate that nov could be involved in the development of Wilms' tumors, and represent a marker of their differentiation state.
Chevalier
G
G
Laboratoire d'oncologie virale et moléculaire, Institut Curie, Orsay, France.
Perbal
B
B
D000818:Animals; D009189:Avian Myeloblastosis Virus / Q000472:pathogenicity*; D002455:Cell Division; D002471:Cell Transformation, Neoplastic / Q000235:genetics; D002642:Chick Embryo; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics; D055513:Connective Tissue Growth Factor; D004195:Disease Models, Animal; D015972:Gene Expression Regulation, Neoplastic; D016162:Genes, Wilms Tumor; D006801:Humans; D017874:Immediate-Early Proteins; D036341:Intercellular Signaling Peptides and Proteins; D008969:Molecular Sequence Data; D055514:Nephroblastoma Overexpressed Protein; D009856:Oncogene Proteins, Viral / Q000235:genetics* / Q000302:isolation & purification / Q000378:metabolism; D011401:Promoter Regions, Genetic; D011518:Proto-Oncogene Proteins / Q000235:genetics*; D014158:Transcription, Genetic; D009396:Wilms Tumor / Q000235:genetics* / Q000473:pathology / Q000821:virology
C526317:CCN2 protein, human; C526319:CCN3 protein, human; D017874:Immediate-Early Proteins; D036341:Intercellular Signaling Peptides and Proteins; D055514:Nephroblastoma Overexpressed Protein; D009856:Oncogene Proteins, Viral; D011518:Proto-Oncogene Proteins; D055513:Connective Tissue Growth Factor