Ontology highlight
ABSTRACT:
SUBMITTER: Wang PY
PROVIDER: S-EPMC5199691 | biostudies-literature | 2017 Jan
REPOSITORIES: biostudies-literature
The Journal of clinical investigation 20161121 1
Li-Fraumeni syndrome (LFS) is a cancer predisposition disorder caused by germline mutations in TP53 that can lead to increased mitochondrial metabolism in patients. However, the implications of altered mitochondrial function for tumorigenesis in LFS are unclear. Here, we have reported that genetic or pharmacologic disruption of mitochondrial respiration improves cancer-free survival in a mouse model of LFS that expresses mutant p53. Mechanistically, inhibition of mitochondrial function increased ...[more]