Journal
PEDIATRIC NEPHROLOGY
Volume 26, Issue 6, Pages 839-852Publisher
SPRINGER
DOI: 10.1007/s00467-010-1689-5
Keywords
Rapamycin; mTORC1; Oncocytoma; Renal cyst; Angiomyolipoma; Tuberous sclerosis complex; Endoplasmic reticulum stress; Autophagy
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Funding
- Department of Defense [TS050008]
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Patients with tuberous sclerosis complex are at great risk of developing renal lesions as part of their disease. These lesions include renal cysts and tumors. Significant advances in understanding the cell biology of these renal lesions has already led to clinical trials demonstrating that pharmacological interventions are likely possible. This review focuses on the pathology of these renal lesions, their underlying cell biology, and the possible therapeutic strategies that may prove to significantly improve care for these patients.
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