scholarly journals Gain of Function of a p53 Hot Spot Mutation in a Mouse Model of Li-Fraumeni Syndrome

Cell ◽  
2004 ◽  
Vol 119 (6) ◽  
pp. 861-872 ◽  
Author(s):  
Gene A. Lang ◽  
Tomoo Iwakuma ◽  
Young-Ah Suh ◽  
Geng Liu ◽  
V.Ashutosh Rao ◽  
...  
Keyword(s):  
Mouse Model ◽  
Hot Spot ◽  
Gain Of Function ◽  
Li Fraumeni Syndrome ◽  
Li Fraumeni

scholarly journals Inhibiting mitochondrial respiration prevents cancer in a mouse model of Li-Fraumeni syndrome

2016 ◽  
Vol 127 (1) ◽  
pp. 132-136 ◽  
Author(s):  
Ping-yuan Wang ◽  
Jie Li ◽  
Farzana L. Walcott ◽  
Ju-Gyeong Kang ◽  
Matthew F. Starost ◽  
...  
Keyword(s):  
Mouse Model ◽  
Mitochondrial Respiration ◽  
Li Fraumeni Syndrome ◽  
Li Fraumeni

scholarly journals Heterogeneity of Li-Fraumeni Syndrome links to unequal gain-of-function effects of p53 mutations

2014 ◽  
Vol 4 (1) ◽  
Author(s):  
Jie Xu ◽  
Jin Qian ◽  
Ye Hu ◽  
Jilin Wang ◽  
Xiaolin Zhou ◽  
...  
Keyword(s):  
P53 Mutations ◽  
Gain Of Function ◽  
Li Fraumeni Syndrome ◽  
Li Fraumeni

scholarly journals Mitochondrial stress delays tumorigenesis in a Li-Fraumeni syndrome mouse model

Cell Stress ◽  
2019 ◽  
Vol 3 (4) ◽  
pp. 115-117 ◽  
Author(s):  
Matthew P. Donnelly ◽  
Jie Li ◽  
Paul M. Hwang
Keyword(s):  
Mouse Model ◽  
Mitochondrial Stress ◽  
Li Fraumeni Syndrome ◽  
Li Fraumeni

scholarly journals Mutant p53 Gain of Function in Two Mouse Models of Li-Fraumeni Syndrome

Cell ◽  
2004 ◽  
Vol 119 (6) ◽  
pp. 847-860 ◽  
Author(s):  
Kenneth P. Olive ◽  
David A. Tuveson ◽  
Zachary C. Ruhe ◽  
Bob Yin ◽  
Nicholas A. Willis ◽  
...  
Keyword(s):  
Mouse Models ◽  
Mutant P53 ◽  
Gain Of Function ◽  
Li Fraumeni Syndrome ◽  
Li Fraumeni

scholarly journals Reducing Fatty Acid Oxidation Improves Cancer-free Survival in a Mouse Model of Li-Fraumeni Syndrome

2020 ◽  
Vol 14 (1) ◽  
pp. 31-40
Author(s):  
Ping-Yuan Wang ◽  
Jin Ma ◽  
Jie Li ◽  
Matthew F. Starost ◽  
Michael J. Wolfgang ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Mouse Model ◽  
Fatty Acid Oxidation ◽  
Acid Oxidation ◽  
Free Survival ◽  
Li Fraumeni Syndrome ◽  
Li Fraumeni

Li-Fraumeni syndrome and adrenal tumours: case report

2018 ◽  
Author(s):  
Mariana Tome ◽  
Jessica Guarino ◽  
Marta Iturregui
Keyword(s):  
Case Report ◽  
Li Fraumeni Syndrome ◽  
Adrenal Tumours ◽  
Li Fraumeni

Neuroradiology Manifestations of Li-Fraumeni Syndrome: Epidemiology, Genetics, Imaging Findings, and Management

Neurographics ◽  
2020 ◽  
Vol 10 (4) ◽  
pp. 228-235
Author(s):  
S. Naganawa ◽  
T. Donohue ◽  
A. Capizzano ◽  
Y. Ota ◽  
J. Kim ◽  
...  
Keyword(s):  
Cell Cycle ◽  
Tumor Suppressor ◽  
Tumor Suppressor Gene ◽  
Soft Tissue Sarcomas ◽  
Suppressor Gene ◽  
Imaging Findings ◽  
Li Fraumeni Syndrome ◽  
Increased Risk ◽  
Li Fraumeni ◽  
Risk Of Cancer

Li-Fraumeni syndrome is a familial cancer predisposition syndrome associated with germline mutation of the tumor suppressor gene 53, which encodes the tumor suppressor p53 protein. Affected patients are predisposed to an increased risk of cancer development, including soft-tissue sarcomas, breast cancer, brain tumors, and adrenocortical carcinoma, among other malignancies. The tumor suppressor gene TP53 plays an important, complex role in regulating the cell cycle, collaborating with transcription factors and other proteins. The disruption of appropriate cell cycle regulation by mutated TP53 is considered to be the cause of tumorigenesis in Li-Fraumeni syndrome. Appropriate surveillance, predominantly by using MR imaging, is used for early malignancy screening in an effort to improve the survival rate among individuals who are affected. Patients with Li-Fraumeni syndrome are also at increased risk for neoplasm development after radiation exposure, and, therefore, avoiding unnecessary radiation in both the diagnostic and therapeutic settings is paramount. Here, we review the epidemiology, genetics, imaging findings, and the current standard surveillance protocol for Li-Fraumeni syndrome from the National Comprehensive Cancer Network as well as potential treatment options.Learning Objective: Describe the cause of second primary malignancy among patients with Li-Fraumeni syndrome.

Spitzoid Melanoma in a Child with Li-Fraumeni Syndrome

2014 ◽  
Vol 17 (1) ◽  
pp. 64-69 ◽  
Author(s):  
Ramya Kollipara ◽  
Linda D. Cooley ◽  
Kimberly A. Horii ◽  
Maxine L. Hetherington ◽  
Philip E. LeBoit ◽  
...  
Keyword(s):  
Li Fraumeni Syndrome ◽  
Li Fraumeni

Utility of interim blood tests for cancer screening in Li-Fraumeni syndrome

2021 ◽  
Author(s):  
Leatrisse Oba ◽  
Ana F. Best ◽  
Phuong L. Mai ◽  
Maria Isabel Achatz ◽  
Paul S. Albert ◽  
...  
Keyword(s):  
Cancer Screening ◽  
Li Fraumeni Syndrome ◽  
Blood Tests ◽  
Li Fraumeni
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