Sklyarova Tatyana, Bonné Stefan, D'Hooge Petra, Denecker Geertrui, Goossens Steven, De Rycke Riet, Borgonie Gaetan, Bösl Michael, van Roy Frans, van Hengel Jolanda
Department for Molecular Biomedical Research, VIB, Ghent, Belgium.
J Invest Dermatol. 2008 Jun;128(6):1375-85. doi: 10.1038/sj.jid.5701189. Epub 2007 Dec 13.
We generated mice deficient in plakophilin-3 (PKP3), a member of the Armadillo-repeat family and a component of desmosomes and stress granules in epithelial cells. In these mice, several subsets of hair follicles (HFs) had morphological abnormalities, and the majority of awl and auchene hair shafts had fewer medullar air columns. Desmosomes were absent from the basal layer of the outer root sheath of HFs and from the matrix cells that are in contact with dermal papillae. In the basal layer of PKP3-null epidermis, densities of desmosomes and adherens junctions were remarkably altered. Compensatory changes in several junctional proteins were observed. PKP3-null mice housed in conventional facilities were prone to dermatitis. Our animal model provides in vivo evidence that PKP3 plays a critical role in morphogenesis of HFs and shafts and in limiting inflammatory responses in the skin.
我们培育出了缺乏桥粒芯蛋白3（PKP3）的小鼠，PKP3是犰狳重复家族的成员，也是上皮细胞中桥粒和应激颗粒的组成部分。在这些小鼠中，几个毛囊亚群出现了形态异常，大多数锥毛和澳米加毛干的髓质气柱较少。毛囊外根鞘的基底层以及与真皮乳头接触的基质细胞中没有桥粒。在PKP3基因缺失的表皮基底层，桥粒和黏着连接的密度发生了显著改变。观察到几种连接蛋白的代偿性变化。饲养在传统设施中的PKP3基因缺失小鼠容易患皮炎。我们的动物模型提供了体内证据，表明PKP3在毛囊和毛干的形态发生以及限制皮肤炎症反应中起关键作用。
