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© 1994 Oxford University Press

OTHER

Localization of the gene whose mutations underlie Hailey—Hailey disease to chromosome 3q

Shigaku Ikeda, Elizabeth A. Welsh1, Anna M. Peluso, Wendy Leyden, Madeleine Duvic2, David T. Woodley3 and Ervin H. Epstein, Jr*

Departments of Dermatology, University of California at San Francisco San Francisco, CA, USA 1Stanford University School of Medicine Stanford, CA, USA 2University of Texas, Houston, Medical School Houston, TX, USA 3Northwestern University Medical School Chicago, IL, USA

*To whom correspondence should be addressed at: San Francisco General Hospital, Building 100, Room 269, 1001 Potrero Street, San Francisco, CA 94110, USA

Received March 29, 1994; Revised May 13, 1994; Accepted May 13, 1994

Hailey—Hailey disease (familial benign chronic pemphigus) is an autosomal dominant skin disease characterized by impaired keratinocyte cohesion and consequent blister formation. In the present study we have used linkage analysis to map the gene for this disease to a region of chromosome 3q between D3S1589 and D3S1316. The maximum combined two point lod score in four families studied was 14.60 at {theta} = 0 at the D3S1290 microsatellite repeat. These findings suggest the presence of a gene not previously known to be involved in keratinocyte cohesion at this site.


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