The role and mechanism of TSC in kidney diseases: a literature review

Abstract Background Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder characterized by multisystem involvement, primarily caused by loss-of-function mutations in the TSC1 or TSC2 genes. TSC is a key integrator of metabolic signaling and cellular stress and has become an impo...

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Main Authors: Qiulin Luo, Xinguo Zheng, Ye Xu, Yuxi Fan, Hedong Zhang, Tengfang Li, Xiangqi Zhang, Longkai Peng, Xin Jiang, Helong Dai
Format: Article
Language:English
Published: BMC 2025-07-01
Series:BMC Nephrology
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Online Access:https://doi.org/10.1186/s12882-025-04260-7
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author Qiulin Luo
Xinguo Zheng
Ye Xu
Yuxi Fan
Hedong Zhang
Tengfang Li
Xiangqi Zhang
Longkai Peng
Xin Jiang
Helong Dai
author_facet Qiulin Luo
Xinguo Zheng
Ye Xu
Yuxi Fan
Hedong Zhang
Tengfang Li
Xiangqi Zhang
Longkai Peng
Xin Jiang
Helong Dai
author_sort Qiulin Luo
collection DOAJ
description Abstract Background Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder characterized by multisystem involvement, primarily caused by loss-of-function mutations in the TSC1 or TSC2 genes. TSC is a key integrator of metabolic signaling and cellular stress and has become an important regulator in several kidney diseases. Summary TSC1 and TSC2 can be used not only as genetic markers for disease diagnosis, but also as potential immunotherapeutic targets for kidney disease. Recent studies on the pathogenesis of TSC may provide guidance for developing new treatment strategies for kidney diseases. Key messages Therefore, we systematically reviewed the molecular biology of TSC and their signaling pathway, regulation of cell metabolism, and immune response in acute renal injury, chronic kidney disease, diabetic kidney disease, renal cysts, benign and malignant intrarenal tumors, and renal angiomyolipomas. We also summarize the efficacy and adverse effects of mTOR inhibitors in the treatment of TSC-related kidney diseases.
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publishDate 2025-07-01
publisher BMC
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series BMC Nephrology
spelling doaj-art-dbce6afb246246fba92a0d54ae50e2532025-08-20T03:03:29ZengBMCBMC Nephrology1471-23692025-07-0126111910.1186/s12882-025-04260-7The role and mechanism of TSC in kidney diseases: a literature reviewQiulin Luo0Xinguo Zheng1Ye Xu2Yuxi Fan3Hedong Zhang4Tengfang Li5Xiangqi Zhang6Longkai Peng7Xin Jiang8Helong Dai9Department of Immunology, School of Basic Medical Science, Central South UniversityDepartment of Immunology, School of Basic Medical Science, Central South UniversityMedical College of Guangxi UniversityDepartment of Immunology, School of Basic Medical Science, Central South UniversityDepartment of Kidney Transplantation, Center of Organ Transplantation, The Second Xiangya Hospital of Central South UniversityDepartment of Kidney Transplantation, Center of Organ Transplantation, The Second Xiangya Hospital of Central South UniversityDepartment of Immunology, School of Basic Medical Science, Central South UniversityDepartment of Kidney Transplantation, Center of Organ Transplantation, The Second Xiangya Hospital of Central South UniversityDepartment of Organ Transplantation, The Fifth Clinical Medical College of Henan, University of Chinese Medicine (Zhengzhou People’s Hospital)Department of Kidney Transplantation, Center of Organ Transplantation, The Second Xiangya Hospital of Central South UniversityAbstract Background Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder characterized by multisystem involvement, primarily caused by loss-of-function mutations in the TSC1 or TSC2 genes. TSC is a key integrator of metabolic signaling and cellular stress and has become an important regulator in several kidney diseases. Summary TSC1 and TSC2 can be used not only as genetic markers for disease diagnosis, but also as potential immunotherapeutic targets for kidney disease. Recent studies on the pathogenesis of TSC may provide guidance for developing new treatment strategies for kidney diseases. Key messages Therefore, we systematically reviewed the molecular biology of TSC and their signaling pathway, regulation of cell metabolism, and immune response in acute renal injury, chronic kidney disease, diabetic kidney disease, renal cysts, benign and malignant intrarenal tumors, and renal angiomyolipomas. We also summarize the efficacy and adverse effects of mTOR inhibitors in the treatment of TSC-related kidney diseases.https://doi.org/10.1186/s12882-025-04260-7Tuberous sclerosis complexKidney diseasesmTOR inhibitorsImmunotherapeutic
spellingShingle Qiulin Luo
Xinguo Zheng
Ye Xu
Yuxi Fan
Hedong Zhang
Tengfang Li
Xiangqi Zhang
Longkai Peng
Xin Jiang
Helong Dai
The role and mechanism of TSC in kidney diseases: a literature review
BMC Nephrology
Tuberous sclerosis complex
Kidney diseases
mTOR inhibitors
Immunotherapeutic
title The role and mechanism of TSC in kidney diseases: a literature review
title_full The role and mechanism of TSC in kidney diseases: a literature review
title_fullStr The role and mechanism of TSC in kidney diseases: a literature review
title_full_unstemmed The role and mechanism of TSC in kidney diseases: a literature review
title_short The role and mechanism of TSC in kidney diseases: a literature review
title_sort role and mechanism of tsc in kidney diseases a literature review
topic Tuberous sclerosis complex
Kidney diseases
mTOR inhibitors
Immunotherapeutic
url https://doi.org/10.1186/s12882-025-04260-7
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