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Mast cell hyperplasia in the skin of Dsg4-deficient hypotrichosis mice, which are long-living mutants of lupus-prone mice

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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-β into intron 8 of Dsg4. Mast cell hyperplasia was observed in the skin of EODhage mice. The abnormally expanded population of lpr T cells, i.e., CD4CD8B220+Thy1.2+ αβT cells, in the splenocytes of EOD mice was reduced in EODhage mice. Therefore, it was suspected that the long-living mutant EODhage 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.

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Acknowledgements

The authors thank Dr. Toshihiko Shiroishi (National Institute of Genetics) for providing MSM mice, Mr. Takuo Hiwatari (Nippon Kayaku Co., Ltd.) and Mr. Kazuumi Kotake (Nippon Kayaku Co., Ltd.) for providing technical assistance in animal experiments, Ms. Naomi Yamaki (Tohoku University) for providing technical assistance in histology, and Ms. Noriko Fujisawa (Tohoku University) and Ms. Emi Yura (Tohoku University) for secretarial assistance. This study is supported by Grants-in-Aid for Scientific Research from the Ministry of Education, Science, Sports, and Culture of Japan to H.F. (# 16790221) and M.O. (# 16390113, 19390108, and 19659096). The authors declare that the experiments comply with the current laws of Japan.

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Correspondence to Hiroshi Furukawa.

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DDBJ accession numbers of the sequences in this article: AB240191, AB240192

MGI accession number of the mutant allele in this article: MGI:3766998

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Zhang, MC., Furukawa, H., Tokunaka, K. et al. Mast cell hyperplasia in the skin of Dsg4-deficient hypotrichosis mice, which are long-living mutants of lupus-prone mice. Immunogenetics 60, 599–607 (2008). https://doi.org/10.1007/s00251-008-0320-4

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  • DOI: https://doi.org/10.1007/s00251-008-0320-4

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