The von Hippel-Lindau tumour suppressor protein: new perspectives

被引：64

作者：

Ohh, M ^{[1
]}

Kaelin, WG

机构：

[1] Harvard Univ, Sch Med, Howard Hughes Med Inst, Boston, MA 02115 USA

[2] Harvard Univ, Sch Med, Dana Farber Canc Inst, Div Adult Oncol, Boston, MA 02115 USA

[3] Harvard Univ, Sch Med, Dept Med, Boston, MA 02115 USA

来源：

MOLECULAR MEDICINE TODAY | 1999年 / 5卷 / 06期

关键词：

D O I：

10.1016/S1357-4310(99)01481-1

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

von Hippel-Lindau (VHL) disease is a hereditary cancer syndrome caused by germline mutations of the VHL tumour suppressor gene. The VHL gene product, pVHL, forms multiprotein complexes that contain elongin B, elongin C and Cul-2, and negatively regulates hypoxia-inducible mRNAs, pVHL is suspected to play a role in ubiquitination given the similarity of elongin C and Cul-2 with Skp1 and Cdc53, respectively, pVHL can also interact with fibronectin and is required for the assembly of a fibronectin matrix, Finally, pVHL, at least indirectly, plays a role in the ability of cells to exit the cell cycle. Thus, pVHL is a tumour suppressor protein that regulates angiogenesis, extracellular matrix formation and the cell cycle.

引用

页码：257 / 263

页数：7

共 47 条

[1] The inducible elongin A elongation activation domain: Structure, function and interaction with the elongin BC complex [J].

Aso, T ;

Haque, D ;

Barstead, RJ ;

Conaway, RC ;

Conaway, JW .

EMBO JOURNAL, 1996, 15 (20) :5557-5566

[2] ELONGIN (SIII) - A MULTISUBUNIT REGULATOR OF ELONGATION BY RNA-POLYMERASE-II [J].

ASO, T ;

LANE, WS ;

CONAWAY, JW ;

CONAWAY, RC .

SCIENCE, 1995, 269 (5229) :1439-1443

[3] SKP1 connects cell cycle regulators to the ubiquitin proteolysis machinery through a novel motif, the F-box [J].

Bai, C ;

Sen, P ;

Hofmann, K ;

Ma, L ;

Goebl, M ;

Harper, JW ;

Elledge, SJ .

CELL, 1996, 86 (02) :263-274

[4] INHIBITION OF TRANSCRIPTION ELONGATION BY THE VHL TUMOR-SUPPRESSOR PROTEIN [J].

DUAN, DR ;

PAUSE, A ;

BURGESS, WH ;

ASO, T ;

CHEN, DYT ;

GARRETT, KP ;

CONAWAY, RC ;

CONAWAY, JW ;

LINEHAN, WM ;

KLAUSNER, RD .

SCIENCE, 1995, 269 (5229) :1402-1406

[5] MUTATIONS OF THE VHL TUMOR-SUPPRESSOR GENE IN RENAL-CARCINOMA [J].

GNARRA, JR ;

TORY, K ;

WENG, Y ;

SCHMIDT, L ;

WEI, MH ;

LI, H ;

LATIF, F ;

LIU, S ;

CHEN, F ;

DUH, FM ;

LUBENSKY, I ;

DUAN, DR ;

FLORENCE, C ;

POZZATTI, R ;

WALTHER, MM ;

BANDER, NH ;

GROSSMAN, HB ;

BRAUCH, H ;

POMER, S ;

BROOKS, JD ;

ISAACS, WB ;

LERMAN, MI ;

ZBAR, B ;

LINEHAN, WM .

NATURE GENETICS, 1994, 7 (01) :85-90

[6] Defective placental vasculogenesis causes embryonic lethality in VHL-deficient mice [J].

Gnarra, JR ;

Ward, JM ;

Porter, FD ;

Wagner, JR ;

Devor, DE ;

Grinberg, A ;

EmmertBuck, MR ;

Westphal, H ;

Klausner, RD ;

Linehan, WM .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1997, 94 (17) :9102-9107

[7] Post-transcriptional regulation of vascular endothelial growth factor mRNA by the product of the VHL tumor suppressor gene [J].

Gnarra, JR ;

Zhou, SB ;

Merrill, MJ ;

Wagner, JR ;

Krumm, A ;

Papavassiliou, E ;

Oldfield, EH ;

Klausner, RD ;

Linehan, WM .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1996, 93 (20) :10589-10594

[8]

Gorospe M, 1999, MOL CELL BIOL, V19, P1289

[9] INTEGRINS - VERSATILITY, MODULATION, AND SIGNALING IN CELL-ADHESION [J].

HYNES, RO .

CELL, 1992, 69 (01) :11-25

[10] Negative regulation of hypoxia-inducible genes by the von Hippel Lindau protein [J].

Iliopoulos, O ;

Levy, AP ;

Jiang, C ;

Kaelin, WG ;

Goldberg, MA .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1996, 93 (20) :10595-10599

← 1 2 3 4 5 →