Multiple roles of the tuberous sclerosis complex genes
- 8 October 2003
- journal article
- review article
- Published by Wiley in Genes, Chromosomes and Cancer
- Vol. 38 (4) , 368-375
- https://doi.org/10.1002/gcc.10256
Abstract
Soon after proposing the “two‐hit” hypothesis for tumorigenesis, Knudson pursued further experimental validation of the concept by using a rat model of dominantly inherited renal tumor. Today, the Eker rat is one of the best characterized models of tuberous sclerosis complex (TSC) and has been used extensively for study of the function of the TSC2 tumor suppressor gene. Along with TSC1, these two genes behave as expected for tumor suppressor genes with evidence for loss of heterozygosity in tumors and suppression of growth when expressed in proliferating cells. Despite much experimental work, the mechanisms of these genes have remained elusive until recently. This review summarizes some of the current concepts in our understanding of the biological and biochemical function of the TSC genes.Keywords
This publication has 67 references indexed in Scilit:
- Tuberin, the tuberous sclerosis complex 2 tumor suppressor gene product, regulates Rho activation, cell adhesion and migrationOncogene, 2002
- Clinical development of mammalian target of rapamycin inhibitorsHematology/Oncology Clinics of North America, 2002
- The DIX domain targets dishevelled to actin stress fibres and vesicular membranesNature, 2002
- Phosphatidylinositol 3-Kinase/Akt Pathway Regulates Tuberous Sclerosis Tumor Suppressor Complex by Phosphorylation of TuberinJournal of Biological Chemistry, 2002
- Rapamycin increases the cellular concentration of the BCL-2 protein and exerts an anti-apoptotic effectEuropean Journal Of Cancer, 2001
- Tuberin Phosphorylation Regulates Its Interaction with HamartinPublished by Elsevier ,2001
- The tuberous sclerosis-1 (TSC1) gene product hamartin suppresses cell growth and augments the expression of the TSC2 product tuberin by inhibiting its ubiquitinationOncogene, 2000
- New developments in the neurobiology of the tuberous sclerosis complexNeurology, 1999
- Embryonic neuronal markers in tuberous sclerosis: Single-cell molecular pathologyProceedings of the National Academy of Sciences, 1996
- Apparent preferential loss of heterozygosity atTSC2 overTSC1 chromosomal region in tuberous sclerosis hamartomasGenes, Chromosomes and Cancer, 1996