Pharmacological blockade of Bcl-2, Bcl-x(L) and Bcl-w by the BH3 mimetic ABT-737 has only minor impact on tumour development in p53-deficient mice.
Cell Death Differ
; 19(4): 623-32, 2012 Apr.
Article
in En
| MEDLINE
| ID: mdl-21997189
The tumour suppressor p53 transcriptionally regulates a range of target genes that control cell growth and survival. Mutations of p53 have been implicated in the development of approximately 50% of human cancers, including those instigated by exposure to mutagens. Although numerically rare, cancers can arise as a consequence of inherited mutations, such as in the Li-Fraumeni syndrome, which is caused by mutation of one p53 allele. Gene-targeted mice deficient for p53 have been generated to study this familial cancer syndrome. On a C57BL/6 background, p53-deficient mice develop primarily thymic lymphoma and more rarely sarcoma. Evasion of apoptosis is considered to be essential for neoplastic transformation. As proteins of the Bcl-2 family are the critical regulators of apoptosis, we investigated the role of the pro-survival members Bcl-2, Bcl-x(L) and Bcl-w in cancer development in p53(+/-) and p53(-/-) mice by testing whether ABT-737, a pharmacological inhibitor of these proteins, could prevent or delay tumourigenesis. Our studies showed that ABT-737 prophylaxis only caused a minor delay and reduction in γ-radiation-induced thymic lymphoma development in p53(-/-) mice, but this was accompanied by a concomitant increase in sarcoma. These data show that, collectively, Bcl-2, Bcl-x(L) and Bcl-w have only minor roles in thymic lymphoma development elicited by defects in p53, and this may indicate that Mcl-1 and/or A1 may feature more prominently in this process.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Sulfonamides
/
Thymus Neoplasms
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Biphenyl Compounds
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Neoplastic Syndromes, Hereditary
/
Proteins
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Tumor Suppressor Protein p53
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Biomimetic Materials
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Bcl-X Protein
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Lymphoma
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Neoplasms, Radiation-Induced
Limits:
Animals
/
Humans
Language:
En
Journal:
Cell Death Differ
Year:
2012
Document type:
Article
Affiliation country:
Australia
Country of publication:
United kingdom