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Publication : Plakophilin-3-deficient mice develop hair coat abnormalities and are prone to cutaneous inflammation.

First Author  Sklyarova T Year  2008
Journal  J Invest Dermatol Volume  128
Issue  6 Pages  1375-85
PubMed ID  18079750 Mgi Jnum  J:136451
Mgi Id  MGI:3796344 Doi  10.1038/sj.jid.5701189
Citation  Sklyarova T, et al. (2008) Plakophilin-3-deficient mice develop hair coat abnormalities and are prone to cutaneous inflammation. J Invest Dermatol 128(6):1375-85
abstractText  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.
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