An Insight of Scientific Developments in TSC for Better Therapeutic Strategy

Tuberous sclerosis complex (TSC) is a rare genetic disease, which is characterized by noncancerous tumors in multi-organ systems in the body. Mutations in the TSC1 or TSC2 genes are known to cause the disease. The resultant mutant proteins TSC1 (hamartin) and TSC2 (tuberin) complex evade its normal...

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Bibliographic Details
Published in:Current topics in medicinal chemistry Vol. 20; no. 23; p. 2080
Main Authors: Natarajan, Nalini, Thiruvenkatam, Vijay
Format: Journal Article
Language:English
Published: United Arab Emirates 01.01.2020
Subjects:
Drug Development
Genes, Tumor Suppressor - drug effects
Humans
Immunosuppressive Agents - chemistry
Immunosuppressive Agents - pharmacology
Sirolimus - chemistry
Sirolimus - pharmacology
Tuberous Sclerosis - drug therapy
Tuberous Sclerosis - metabolism
Tuberous Sclerosis Complex 1 Protein - antagonists & inhibitors
Tuberous Sclerosis Complex 1 Protein - genetics
Tuberous Sclerosis Complex 1 Protein - metabolism
Tuberous Sclerosis Complex 2 Protein - antagonists & inhibitors
Tuberous Sclerosis Complex 2 Protein - genetics
Tuberous Sclerosis Complex 2 Protein - metabolism
mTOR inhibition
Coiled-coil sequence
Protein-protein interaction
Disruptive mutation
Heat shock proteins
Hypoxia
Rapalogs treatment
Tuberous sclerosis complex
Crystal structure
ISSN:1873-4294, 1873-4294
Online Access:Get more information
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Summary:Tuberous sclerosis complex (TSC) is a rare genetic disease, which is characterized by noncancerous tumors in multi-organ systems in the body. Mutations in the TSC1 or TSC2 genes are known to cause the disease. The resultant mutant proteins TSC1 (hamartin) and TSC2 (tuberin) complex evade its normal tumor suppressor function, which leads to abnormal cell growth and proliferation. Both TSC1 and TSC2 are involved in several protein-protein interactions, which play a significant role in maintaining cellular homeostasis. The recent biochemical, genetic, structural biology, clinical and drug discovery advancements on TSC give a useful insight into the disease as well as the molecular aspects of TSC1 and TSC2. The complex nature of TSC disease, a wide range of manifestations, mosaicism and several other factors limits the treatment choices. This review is a compilation of the course of TSC, starting from its discovery to the current findings that would take us a step ahead in finding a cure for TSC.
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ISSN:1873-4294
1873-4294
DOI:10.2174/1568026620666200825170355