Multiple roles of the tuberous sclerosis complex genes

被引:22
作者
Yeung, RS [1 ]
机构
[1] Univ Washington, Dept Surg, Seattle, WA 98195 USA
关键词
D O I
10.1002/gcc.10256
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
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. (C) 2003 Wiley-Liss, Inc.
引用
收藏
页码:368 / 375
页数:8
相关论文
共 76 条
[1]   Tuberin phosphorylation regulates its interaction with hamartin - Two proteins involved in tuberous sclerosis [J].
Aicher, LD ;
Campbell, JS ;
Yeung, RS .
JOURNAL OF BIOLOGICAL CHEMISTRY, 2001, 276 (24) :21017-21021
[2]   Tuberin, the tuberous sclerosis complex 2 tumor suppressor gene product, regulates Rho activation, cell adhesion and migration [J].
Astrinidis, A ;
Cash, TP ;
Hunter, DS ;
Walker, CL ;
Chernoff, J ;
Henske, EP .
ONCOGENE, 2002, 21 (55) :8470-8476
[3]   The tuberous sclerosis-1 (TSC1) gene product hamartin suppresses cell growth and augments the expression of the TSC2 product tuberin by inhibiting its ubiquitination [J].
Benvenuto, G ;
Li, SW ;
Brown, SJ ;
Braverman, R ;
Vass, WC ;
Cheadle, JP ;
Halley, DJJ ;
Sampson, JR ;
Wienecke, R ;
DeClue, JE .
ONCOGENE, 2000, 19 (54) :6306-6316
[4]  
Bourneville D., 1880, ARCH NEUROL-PARIS, V1, P81
[5]   Rapamycin increases the cellular concentration of the BCL-2 protein and exerts an anti-apoptotic effect [J].
Calastretti, A ;
Rancati, F ;
Ceriani, MC ;
Asnaghi, L ;
Canti, G ;
Nicolin, A .
EUROPEAN JOURNAL OF CANCER, 2001, 37 (16) :2121-2128
[6]   The DIX domain targets dishevelled to actin stress fibres and vesicular membranes [J].
Capelluto, DGS ;
Kutateladze, TG ;
Habas, R ;
Finkielstein, CV ;
He, X ;
Overduin, M .
NATURE, 2002, 419 (6908) :726-729
[7]  
Carbonara C, 1996, GENE CHROMOSOME CANC, V15, P18, DOI 10.1002/(SICI)1098-2264(199601)15:1<18::AID-GCC3>3.0.CO
[8]  
2-7
[9]   Embryonic neuronal markers in tuberous sclerosis: Single-cell molecular pathology [J].
Crino, PB ;
Trojanowski, JQ ;
Dichter, MA ;
Eberwine, J .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1996, 93 (24) :14152-14157
[10]   New developments in the neurobiology of the tuberous sclerosis complex [J].
Crino, PB ;
Henske, EP .
NEUROLOGY, 1999, 53 (07) :1384-1390