p63, a Story of Mice and Men

被引：149

作者：

Vanbokhoven, Hans ^{[3
]}

Melino, Gerry ^{[4
,5
]}

Candi, Eleonora ^{[4
]}

Declercq, Wim ^{[1
,2
]}

机构：

[1] VIB, Mol Signaling & Cell Death Unit, Dept Mol Biomed Res, Ghent, Belgium

[2] Univ Ghent, Dept Biomed Mol Biol, B-9000 Ghent, Belgium

[3] Radboud Univ Nijmegen, Med Ctr, Dept Human Genet, Nijmegen Ctr Mol Life Sci,Mol Neurogenet Unit, NL-6525 ED Nijmegen, Netherlands

[4] Univ Roma Tor Vergata, Dept Expt Med & Biochem Sci, IDI IRCCS Biochem Lab, I-00173 Rome, Italy

[5] Univ Leicester, Med Res Council, Toxicol Unit, Leicester, Leics, England

来源：

JOURNAL OF INVESTIGATIVE DERMATOLOGY | 2011年 / 131卷 / 06期

关键词：

HAND/SPLIT-FOOT MALFORMATION; HODGKIN ECTODERMAL DYSPLASIA; PROTECTS MOUSE OOCYTES; HAY-WELLS-SYNDROME; RAPP-HODGKIN; CLEFT-LIP; STEM-CELLS; IKK-ALPHA; EPIDERMAL DEVELOPMENT; LIMB DEVELOPMENT;

D O I：

10.1038/jid.2011.84

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100227 [皮肤病学];

摘要：

The transcription factor p63 is essential for the formation of the epidermis and other stratifying epithelia. This is clearly demonstrated by the severe abnormality of p63-deficient mice and by the development of certain types of ectodermal dysplasias in humans as a result of p63 mutations. Investigation of the in vivo functions of p63 is complicated by the occurrence of 10 different splicing isoforms and by its interaction with the other family members, p53 and p73. In vitro and in vivo models have been used to unravel the functions of p63 and its different isoforms, but the results or their interpretation are often contradictory. This review focuses on what mammalian in vivo models and patient studies have taught us in the last 10 years.

引用

页码：1196 / 1207

页数：12

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