PMID: 8941678Dec 1, 1996Paper

Hailey-Hailey disease keratinocytes: normal assembly of cell-cell junctions in vitro

The Journal of Investigative Dermatology
J E CooleyE J O'Keefe

Abstract

The blisters in the inherited disorder, Hailey-Hailey disease, may be caused by defective epidermal junctional complexes. We evaluated these structural complexes in vivo and in vitro. We induced a vesicular lesion in the apparently normal skin of a patient with Hailey-Hailey disease and studied a biopsy of this lesion by transmission electron microscopy. To determine whether acantholysis was related to a defect in the number or assembly of intercellular junctions, we cultured Hailey-Hailey disease keratinocytes in medium containing 0.1 mM Ca2+ and increased the [Ca2+] to 1.1 mM in order to induce assembly of cell-cell junctions. Keratinocytes were examined by double immunofluorescence with antibodies to the desmosome protein, desmoplakin, and the adherens junction protein, vinculin, at intervals after the increase in [Ca2+]. Characteristic Hailey-Hailey disease histopathology was observed by electron microscopy of the patient's skin after trauma, but we found no splitting of desmosomes. Based on the location, intensity, and rate of change of immunofluorescent staining, Hailey-Hailey and normal keratinocytes did not differ in their ability to assemble desmosomes and adherens junctions. Furthermore, we observed no significant mor...Continue Reading

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Citations

Jul 1, 2015·Experimental Dermatology·Mitsuhiro MatsudaTakashi Hashimoto
Feb 18, 2014·Skin Research and Technology : Official Journal of International Society for Bioengineering and the Skin (ISBS) [and] International Society for Digital Imaging of Skin (ISDIS) [and] International Society for Skin Imaging (ISSI)·M KurzejaM Olszewska
Dec 21, 2006·Journal of Cosmetic Dermatology·Cheryl BansalThomas E Rohrer
Mar 11, 2008·Skinmed·Yonit WohlSarah Brenner
Nov 25, 2003·The Journal of Investigative Dermatology·Ida AronchikTheodora Mauro

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