Increased oxidative metabolism in the Li-Fraumeni syndrome.

  title={Increased oxidative metabolism in the Li-Fraumeni syndrome.},
  author={Ping-yuan Wang and Wenzhe Ma and Joon-Young Park and Francesco Saverio Celi and Ross Arena and Jeong Woo Choi and Qais Abu Ali and Dotti J Tripodi and Jie Zhuang and Cory U. Lago and Louise C. Strong and S. Lalith Talagala and Robert S Balaban and Ju-Gyeong Kang and Paul M. Hwang},
  journal={The New England journal of medicine},
  volume={368 11},
There is growing evidence that alterations in metabolism may contribute to tumorigenesis. Here, we report on members of families with the Li-Fraumeni syndrome who carry germline mutations in TP53, the gene encoding the tumor-suppressor protein p53. As compared with family members who are not carriers and with healthy volunteers, family members with these mutations have increased oxidative phosphorylation of skeletal muscle. Basic experimental studies of tissue samples from patients with the Li… CONTINUE READING
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