title: Plakophilin-3-deficient mice develop hair coat abnormalities and are prone to cutaneous inflammation.
abstract: 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.
authors: Department for Molecular Biomedical Research, VIB, Ghent, Belgium.
authors: Tatyana
authors: T
authors: Sklyarova
authors: Stefan
authors: S
authors: Bonné
authors: Petra
authors: P
authors: D'Hooge
authors: Geertrui
authors: G
authors: Denecker
authors: Steven
authors: S
authors: Goossens
authors: Riet
authors: R
authors: De Rycke
authors: Gaetan
authors: G
authors: Borgonie
authors: Michael
authors: M
authors: Bösl
authors: Frans
authors: F
authors: van Roy
authors: Jolanda
authors: J
authors: van Hengel
mesh_terms: D000505:Alopecia / Q000235:genetics / Q000473:pathology; D000818:Animals; D017209:Apoptosis; D003872:Dermatitis / Q000378:metabolism; D003896:Desmosomes / Q000378:metabolism; D004817:Epidermis / Q000473:pathology; D005786:Gene Expression Regulation; D006201:Hair Diseases / Q000235:genetics / Q000473:pathology*; D007249:Inflammation; D051379:Mice; D008810:Mice, Inbred C57BL; D008957:Models, Genetic; D009154:Mutation; D051191:Plakophilins / Q000235:genetics* / Q000502:physiology*; D012867:Skin / Q000276:immunology / Q000473:pathology*
chemical_list: D051191:Plakophilins