The effects of a mutant connexin 26 on epidermal differentiation.
|
| Abstract |
To elucidate the mode of action of dominant mutant connexins in causing inherited skin diseases, transgenic mice were produced that express the true Vohwinkel syndrome-associated mutant Cx26 (D66H), from a keratin 10 promoter, specifically in the suprabasal epidermal keratinocytes. Following birth, the transgenic mice developed keratoderma similar to that of human carriers of Cx26 (D66H). Expression of the transgene resulted in a loss of Cx26 and Cx30 at intercellular junctions of epidermal keratinocytes and accumulation of these connexins in the cytoplasm. Injection of primary mouse keratinocytes with Lucifer Yellow showed no difference in terms of dye spreading between transgenic and non transgenic keratinocytes in vitro. Expression of the mutant Cx26 (D66H) did not interfere with the formation of the epidermal water barrier during late embryonic development. Attempts to produce transgenic mice expressing the wild type form of Cx26 from the K10 promoter failed to produce viable animals although transgenic embryos were recovered at days 9 and 12 of gestation, suggesting that the transgene might be embryonic lethal.
|
|---|---|
| Authors | George Bakirtzis, Susan Jamieson, Trond Aasen, Sheila Bryson, Stephen Forrow, Laurence Tetley, Malcolm Finbow, David Greenhalgh, Malcolm Hodgins |
| Journal | Cell communication & adhesion (Cell Commun Adhes) 2003 Jul-Dec Vol. 10 Issue 4-6 Pg. 359-64 ISSN: 1541-9061 [Print] England |
| PMID | 14681042 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't) |
| Chemical References |
|
| Topics |
|
Join CureHunter, for free Research Interface BASIC access!
Realize the full power of the drug-disease research graph!