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Tight junctions in Hailey-Hailey and Darier's diseases
Laura Raiko1, Pekka Leinonen2, Päivi M Hägg3
1Department of Dermatology, University of Turku and Turku University Central Hospital, Turku, Finland;
Tight junctions (TJ) maintain skin barrier function. In Hailey-Hailey disease and Darier's disease, TJ proteins show altered distribution, impacting epidermal barrier integrity and increasing water loss.
Area of Science:
- Dermatology
- Cell Biology
- Biochemistry
Background:
- Hailey-Hailey disease (HHD) and Darier's disease (DD) involve desmosomal disruption and acantholysis.
- Tight junctions (TJ) are crucial for epidermal barrier function.
- Altered epidermal differentiation can affect TJ component expression.
Purpose of the Study:
- To investigate the expression and dynamics of TJ proteins during desmosome disruption in HHD and DD.
- To evaluate the epidermal barrier function in HHD and DD lesions.
Main Methods:
- Indirect immunofluorescence and avidin-biotin labeling for junctional proteins.
- Confocal laser scanning microscopy on HHD and DD skin samples.
- Measurement of transepidermal water loss (TEWL) in lesional and normal skin.
Main Results:
- TJ proteins (claudin-1, claudin-4, ZO-1, occludin) showed altered localization in HHD lesions, with ZO-1 expanding to acantholytic cells.
- Claudin-1 and ZO-1 persisted at cell-cell contacts in acantholytic cells.
- Increased TEWL indicated impaired epidermal barrier function in lesional skin.
Conclusions:
- TJ components exhibit distinct dynamics during acantholysis in HHD and DD compared to desmosomal and adherens junction proteins.
- Altered TJ dynamics contribute to the compromised epidermal barrier in HHD and DD.
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