Reduced expression of the antiapoptotic proteins of Bcl-2 gene family in the lesional epidermis of patients with Darier’s disease
Article first published online: 6 OCT 2006
Journal of Cutaneous Pathology
Volume 34, Issue 3, pages 234–238, March 2007
How to Cite
Pasmatzi, E., Badavanis, G., Monastirli, A. and Tsambaos, D. (2007), Reduced expression of the antiapoptotic proteins of Bcl-2 gene family in the lesional epidermis of patients with Darier’s disease. Journal of Cutaneous Pathology, 34: 234–238. doi: 10.1111/j.1600-0560.2006.00600.x
- Issue published online: 6 OCT 2006
- Article first published online: 6 OCT 2006
- Accepted for publication March 17, 2006
Background: Dyskeratotic cells in Darier’s disease (DD) are thought to represent apoptotic keratinocytes. Bcl-2 gene family proteins play a major role in the regulation of apoptosis of epidermal keratinocytes and reveal pleiotropic interactions with intracellular Ca2+ homeostasis. The latter is impaired in DD because of mutations of ATP2A2 gene that encodes the type 2 isoforms of the sarcoplasmic/endoplasmic reticulum (ER) Ca++ ATPase 2 (SERCA2) pump.
Methods: The expression of Bcl-2, Bax, and Bcl-xL proteins was investigated in the epidermis of 11 patients with DD and of 11 sex- and age-matched healthy controls by immunohistochemistry.
Results: The expression of Bcl-2 and Bcl-xL was clearly reduced in the lesional epidermis of the patients, as compared with the normal epidermis of healthy controls, whereas the expression of Bax remained unaltered.
Conclusions: The alterations in the expression of Bcl-2 gene family proteins could be a crucial event for the activation of the apoptotic process in the lesional epidermis of DD patients and for the occurrence of the characteristic dyskeratotic keratinocytes. The question as to whether these alterations are associated with the ER Ca2+ depletion in DD or represent secondary phenomena unrelated to the genetic defect of this genodermatosis remains to be elucidated.