Tuberous sclerosis complex (TSC) is characterized by hamartoma formation in a wide range of tissues. TSC tumors grow in kidney, brain, skin, and even in heart. The TSC disease is caused by mutation in either the TSC1 or TSC2 tumor suppressor gene. Mutations in TSC2 also cause lymphangioleiomyomatosis, a progressive lung disease that affects women. The TSC tumor cells die easily in the absence of glucose. p53 is the most important tumor suppressor gene in human cancers. Inactivation of p53 is found in 50% of all human cancers. Preliminary study from our laboratory has indicated a possible link between TSC1/TSC2 and p53. The goal of this project is to investigate the function of p53 in glucose starvation-induced cell death in TSC tumor cells. A functional connection between TSC1/TSC2 and p53 will help our understanding TSC tumor growth. Moreover, such information may be useful for future treatment of TSC disease. |
