Altered cell differentiation and proliferation in mice lacking p57(KIP2) indicates a role in Beckwith-Wiedemann syndrome

被引：632

作者：

Zhang, PM

Liegeois, NJ

Wong, C

Finegold, M

Hou, H

Thompson, JC

Silverman, A

Harper, JW

DePinho, RA

Elledge, SJ

机构：

[1] YESHIVA UNIV ALBERT EINSTEIN COLL MED,DEPT MICROBIOL & IMMUNOL,BRONX,NY 10461

[2] BAYLOR COLL MED,VERNA & MARRS MCLEAN DEPT BIOCHEM,HOUSTON,TX 77030

[3] BAYLOR COLL MED,HOWARD HUGHES MED INST,HOUSTON,TX 77030

[4] BAYLOR COLL MED,DEPT PATHOL,HOUSTON,TX 77030

[5] BAYLOR COLL MED,DEPT MOL & HUMAN GENET,HOUSTON,TX 77030

来源：

NATURE | 1997年 / 387卷 / 6629期

关键词：

D O I：

10.1038/387151a0

中图分类号：

O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];

学科分类号：

07 ; 0710 ; 09 ;

摘要：

Mice lacking the imprinted Cdk inhibitor p57(KIP2) have altered cell proliferation and differentiation, leading to abdominal muscle defects; cleft palate; endochondral bone ossification defects with incomplete differentiation of hypertrophic chondrocytes; renal medullary dysplasia; adrenal cortical hyperplasia and cytomegaly; and lens cell hyperproliferation and apoptosis. Many of these phenotypes are also seen in patients with Beckwith-Wiedemann syndrome, a pleiotropic hereditary disorder characterized by overgrowth and predisposition to cancer, suggesting that loss of p57(KIP2) expression may play a role in the condition.

引用

页码：151 / 158

页数：8

共 33 条

[1]

BECKWITH J B, 1969, Birth Defects Original Article Series, V5, P188

[2] Shared role of the pRB-related p130 and p107 proteins in limb development [J].

Cobrinik, D ;

Lee, MH ;

Hannon, G ;

Mulligan, G ;

Bronson, RT ;

Dyson, N ;

Harlow, E ;

Beach, D ;

Weinberg, RA ;

Jacks, T .

GENES & DEVELOPMENT, 1996, 10 (13) :1633-1644

[3] MICE LACKING P21(C/P1/WAF1) UNDERGO NORMAL DEVELOPMENT, BUT ARE DEFECTIVE IN G1 CHECKPOINT CONTROL [J].

DENG, CX ;

ZHANG, PM ;

HARPER, JW ;

ELLEDGE, SJ ;

LEDER, P .

CELL, 1995, 82 (04) :675-684

[4]

DYNLACHT BD, IN PRESS METHODS ENZ

[5]

ELLIOTT M, 1994, CLIN GENET, V46, P168

[6]

FERGUSON MWJ, 1988, DEVELOPMENT, V103, P41

[7] A syndrome of multiorgan hyperplasia with features of gigantism, tumorigenesis, and female sterility in p27(Kip1)-deficient mice [J].

Fero, ML ;

Rivkin, M ;

Tasch, M ;

Porter, P ;

Carow, CE ;

Firpo, E ;

Polyak, K ;

Tsai, LH ;

Broudy, V ;

Perlmutter, RM ;

Kaushansky, K ;

Roberts, JM .

CELL, 1996, 85 (05) :733-744

[8]

Harper JW, 1996, CURR OPIN GENET DEV, V6, P56

[9]

HASTIE ND, 1994, ANNU REV GENET, V28, P523

[10] An imprinted gene p57(KIP2) is mutated in Beckwith-Wiedemann syndrome [J].

Hatada, I ;

Ohashi, H ;

Fukushima, Y ;

Kaneko, Y ;

Inoue, M ;

Komoto, Y ;

Okada, A ;

Ohishi, S ;

Nabetani, A ;

Morisaki, H ;

Nakayama, M ;

Niikawa, N ;

Mukai, T .

NATURE GENETICS, 1996, 14 (02) :171-173

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