The WT1 gene encodes a zinc finger transcription factor important for normal kidney development. WT1 is a suppressor for Wilms tumour development and an oncogene for diverse malignant tumours. We recently established cell lines from primary Wilms tumours with different WT1 mutations. To investigate the function of mutant WT1 proteins, we performed WT1 knockdown experiments in cell lines with a ...

The Wilms' tumor suppressor gene, WT1, encodes a zinc finger transcription factor that has been demonstrated to negatively regulate several growth factor and cognate receptor genes. However, inconsistent with its tumor suppressor function, WT1 has also been demonstrated to be required to inhibit programmed cell death in vitro and in vivo. Moreover, anaplastic Wilms' tumors, which typically expr...

BACKGROUND The most common renal tumors in adults is renal cell carcinoma. Wilms' tumor in subjects older than 16 years is rare; only 3% of Wilms' tumors are reported in adults, which explain the difficulties in diagnosis and treatment of this tumor entity in this age group. METHODS Patient with stage IV adult nephroblastoma with favorable histology was described, current treatment modalities...

Transcription factor Wilms’ tumor 1 (WT1) was originally identified as a tumor suppressor for Wilms’ tumor, but it is also overexpressed in a variety of cancer cells, suggesting a potential oncogenic function of WT1. It is important to understand molecular mechanisms underlying these dual functions of WT1 in tumorigenesis. In the current study, we report a synergistic role for signal transducer...

Mutations of the p53 tumor suppressor gene occur frequently in a variety of adult-onset tumors, including colon, breast, lung, and brain, yet are infrequently identified in pediatric malignancies. Wilms' tumor, a common solid tumor of childhood, can be associated with mutations of the WT1 gene. Alterations of the p53 gene have been shown to modulate the ability of WT1 to transactivate its targe...

Loss of imprinting at insulin-like growth factor II (IGFII), in association with H19 silencing, has been described previously in a subgroup of Beckwith-Wiedemann syndrome (BWS) patients who have an elevated risk for Wilms' tumor. An equivalent somatic mutation occurs in sporadic Wilms' tumor. We describe a family with overgrowth in three generations and Wilms' tumor in two generations, with pat...

how to cite this article: talebian a, goudarzi rm, mohammadzadeh m , mirzadeh as. vincristine-induced cranial neuropathy. iran j child neurol. 2014 winter; 8(1):66-68.   abstract vincristine (vcr) is a vinca alkaloid that is used for treatment of many malignancies. the vinca alkaloids are neurotoxic, usually causing a peripheral neuropathy, but cranial neuropathies are rare as side effects.  de...

epidermal nevus syndrome is a rare congenital disorder, characterized by epidermal nevi and multiple organ involvement. multicystic kidney disease has been very rarely reported in this syndrome. here is the report of a boy presented with multiple epidermal nevi, cardiac anomaly, seizure attack, hemi hypertrophy, and multicystic dysplastic kidney complicated with wilms' tumor. according to this ...

Book details Willemijn Wilms Floet URBAN OASES Dutch Hofjes as Hidden Architectural Gems . nai010 publishers, 2021. ISBN 978-94-6208-660-9. € 39.95, paperback (Also available in and e-book English Dutch). pp. 208, with illustrations (220 full color).

at an early age. Furthermore, a genetic predisposition to develop the tumour is associated with aniridia, genitourinary abnormalities and mental retardation (the WAGR syndrome) (1). Children with this rare syndrome typically carry a germline deletion involving band p13 on one of the two (parentally-derived) chromosome 11 homologues (2). The inherited 11 p deletion in WAGR and hereditary Wilms' ...