Resumen

HERNANDEZ FERNANDEZ, Rolando A. Wilm's toumor. Rev haban cienc méd [online]. 2011, vol.10, n.2. ISSN 1729-519X.

Wilm's tumor is the most frequent cancer of the kidney in childhood with onset before five year old. WT gene was mapped in 11p13 and span 50 kb with 10 exons. WT modifications would be either genetics or epigenetics. The gene code for at least 24 isoforms of protein products. Other chromosomes loci whose alterations may be cause of disease have been identified, so Wilm's tumor is a remarkable paradigm of genetic heterogeneity. In this paper an analysis of the molecular basis of the disease is presented and, also, a brief references on main hereditary syndromes which include Wilm's tumor.

Palabras clave : Wilm's tumor; genetic heterogeneity; genomic imprinting; Denys-Drash syndrome; WAGR syndrome; Beckwith-Wiedeman syndrome; Simpson-Golabi-Behmel syndrome; Frasier syndrome.

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