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• W2054137427 abstract "Summary The TP 53 (p53) pathway can be inhibited by TP 53 mutation or deletion or by MDM 2 overexpression. Both occur in B urkitt lymphoma ( BL ), but many cases lack either abnormality. Expression patterns of the TP 53 inhibitor MDM 4 have not been reported in BL , and increased MDM 4 could deregulate the TP 53 pathway in cases without TP 53 or MDM 2 abnormalities. We investigated TP 53 pathway disruption in paediatric BL patient samples ( n = 30) by studying MDM 4, MDM 2, and CDKN 1 A (p21) protein and m RNA expression; TP 53 mutations; TP 53 protein expression; and gene copy number abnormalities. MDM 4 protein was expressed in 30/30 tumours, and MDM 2 protein was weakly expressed in 7/30 (23%). All cases were negative for CDKN 1 A protein, and CDKN 1 A m RNA levels were decreased. TP 53 mutations were detected in 5/28 (18%) cases and confirmed by sequencing. TP 53 protein was expressed in 15/30 (50%) cases, including 7/8 with TP 53 genetic alterations. MDM 2 protein and m RNA expression levels did not correlate with lack of TP 53 genetic changes or TP 53 protein expression; however, there was an inverse relationship between detectable TP 53 protein expression and MDM 4 copy number gains and m RNA expression. The TP 53 pathway is deregulated in paediatric BL cases, and increased MDM 4 expression may be the primary mechanism in some cases." @default.
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• W2054137427 date "2012-07-27" @default.
• W2054137427 modified "2023-09-27" @default.
• W2054137427 title "TP53 pathway analysis in paediatric Burkitt lymphoma reveals increased MDM4 expression as the only TP53 pathway abnormality detected in a subset of cases" @default.
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• W2054137427 doi "https://doi.org/10.1111/j.1365-2141.2012.09243.x" @default.
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