p53 and Medulloblastoma
نویسندگان
چکیده
Vijay Ramaswamy,1,2,3 Carolina Nör,3,4 and Michael D. Taylor2,3,4 Division of Haematology/Oncology, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario M5S 1A1, Canada Program in Developmental and Stem Cell Biology, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada Division of Neurosurgery, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada
منابع مشابه
Differential forms of p53 in medulloblastoma primary tumors, cell lines and xenografts.
Medulloblastoma is the most common malignant brain tumor in childhood. The most prevalent chromosomal abnormalities are isochromosome 17q and loss of 17p, the location of the tumor suppressor gene p53. Mutations in the p53 gene in medulloblastoma are relatively infrequent but have recently been correlated to poor prognosis. Furthermore, the p53 gene encodes nine different isoforms, which may ha...
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Many studies have been focused on cytogenetic and molecular genetic defects in brain tumors; therefore the role of ATM as a tumor suppressor gene in these tumors is poorly considered. In this study mutation analysis of exon 19 and 39 of ATM gene and P53 accumulation were investigated by PCR-SSCP, sequencing, and flow cytometry . Four polymorphisms including D1853N, IVS 38-8 T?C, F858L, P872T we...
متن کاملLoss of p53 but not ARF accelerates medulloblastoma in mice heterozygous for patched.
Brain malignancies represent the most common solid tumors in children, and they are responsible for significant mortality and morbidity. The molecular basis of the most common malignant pediatric brain tumor, medulloblastoma, is poorly understood. Mutations in several genes including the human homologue of the Drosophila segment polarity gene, patched (PTCH), the adenomatous polyposis coli gene...
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Substantial neural defects are often present in mice with targeted inactivation of DNA repair factors such as DNA ligase IV (Lig4). Whereas Lig4(-/-) mice undergo widespread neural apoptosis and die during development, p53 deficiency rescues this death. We found that all Lig4(-/-)p53(-/-) mice developed medulloblastoma, but did not develop other tumors of the nervous system. Lig4(-/-)p53(-/-) m...
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We undertook a comprehensive clinical and biological investigation of serial medulloblastoma biopsies obtained at diagnosis and relapse. Combined MYC family amplifications and P53 pathway defects commonly emerged at relapse, and all patients in this group died of rapidly progressive disease postrelapse. To study this interaction, we investigated a transgenic model of MYCN-driven medulloblastoma...
متن کاملAnalysis of a mutant p53 protein arising in a medulloblastoma from a mouse transgenic for the JC virus early region.
BACKGROUND JC virus (JCV) is a polyomavirus that causes progressive multifocal leukoencephalopathy (PML) in humans and is highly oncogenic in experimental animals. Transgenic mice with JCV T-antigen develop cerebellar tumors, which resemble human medulloblastomas, containing two distinct cell subpopulations, T-antigen positive and negative. In T-negative clones, a novel mutant p53 was detected ...
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