title: Mast cell hyperplasia in the skin of Dsg4-deficient hypotrichosis mice, which are long-living mutants of lupus-prone mice.
abstract: Desmosomal cadherins are essential cell adhesion molecules expressed in the epidermis. We identified a mutation of a cadherin superfamily member, namely, desmoglein 4 (Dsg4), in early onset of death (EOD)( hage ) mice with hypotrichosis. The mutation was induced by the insertion of an early transposon II-beta into intron 8 of Dsg4. Mast cell hyperplasia was observed in the skin of EOD( hage ) mice. The abnormally expanded population of lpr T cells, i.e., CD4(-)CD8(-)B220(+)Thy1.2(+) alphabetaT cells, in the splenocytes of EOD mice was reduced in EOD( hage ) mice. Therefore, it was suspected that the long-living mutant EOD( hage ) mice were selected from lupus-prone EOD mice because of their immunological immaturity. These findings clearly indicate that Dsg4 is an important molecule for the formation of hair follicles and hypothesize that unorganized hyperplastic hair follicles in anagen due to the Dsg4 mutation provide niches for mast cell precursors in the skin.
authors.affiliation: Department of Pathology, Tohoku University Graduate School of Medicine, Seiryo-machi 2-1, Aoba-ku, Sendai, Japan , 980-8575.
authors.forename: Ming-Cai
authors.initials: MC
authors.lastname: Zhang
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Furukawa
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Tokunaka
authors.forename: Kan
authors.initials: K
authors.lastname: Saiga
authors.forename: Fumiko
authors.initials: F
authors.lastname: Date
authors.forename: Yuji
authors.initials: Y
authors.lastname: Owada
authors.forename: Masato
authors.initials: M
authors.lastname: Nose
authors.forename: Masao
authors.initials: M
authors.lastname: Ono
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D001483:Base Sequence; D003001:Cloning, Molecular; D051182:Desmogleins / Q000502:physiology*; D005434:Flow Cytometry; D020022:Genetic Predisposition to Disease; D018859:Hair Follicle / Q000276:immunology / Q000473:pathology; D006965:Hyperplasia; D007039:Hypotrichosis / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D007438:Introns / Q000235:genetics; D008177:Lupus Vulgaris / Q000276:immunology / Q000473:pathology*; D008407:Mast Cells / Q000473:pathology*; D051379:Mice; D019463:Mice, Inbred MRL lpr / Q000235:genetics; D018345:Mice, Knockout; D008817:Mice, Mutant Strains / Q000235:genetics; D008969:Molecular Sequence Data; D009154:Mutation / Q000235:genetics*; D017386:Sequence Homology, Amino Acid; D012867:Skin / Q000276:immunology / Q000378:metabolism / Q000473:pathology*; D015996:Survival Rate
chemical_list: D051182:Desmogleins; C475358:Dsg4 protein, mouse