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Hypotonic, Acidic Oxidizing Solution Containing Hypochlorous Acid (HClO) as a Potential Treatment of Hailey-Hailey Disease

Department of Molecular Medicine, Sapienza University of Rome, 00161 Rome, Italy
Center of Life Nano Science Sapienza, Istituto Italiano di Tecnologia, 00161 Rome, Italy
Authors to whom correspondence should be addressed.
Academic Editor: Valeria Amendola
Molecules 2019, 24(24), 4427;
Received: 1 October 2019 / Revised: 29 November 2019 / Accepted: 2 December 2019 / Published: 4 December 2019
(This article belongs to the Special Issue Molecular Recognition and Self-Assembly in Chemistry and Medicine)
Hailey–Hailey disease (HHD) is a rare, chronic and recurrent blistering disorder, characterized by erosions occurring primarily in intertriginous regions and histologically by suprabasal acantholysis. Mutation of the Golgi Ca2+-ATPase ATP2C1 has been identified as having a causative role in Hailey–Hailey disease. HHD-derived keratinocytes have increased oxidative-stress that is associated with impaired proliferation and differentiation. Additionally, HHD is characterized by skin lesions that do not heal and by recurrent skin infections, indicating that HHD keratinocytes might not respond well to challenges such as wounding or infection. Hypochlorous acid has been demonstrated in vitro and in vivo to possess properties that rescue both oxidative stress and altered wound repair process. Thus, we investigated the potential effects of a stabilized form of hypochlorous acid (APR-TD012) in an in vitro model of HHD. We found that treatment of ATP2C1-defective keratinocytes with APR-TD012 contributed to upregulation of Nrf2 (nuclear factor (erythroid-derived 2)-like 2). Additionally, APR TD012-treatment restored the defective proliferative capability of siATP2C1-treated keratinocytes. We also found that the APR-TD012 treatment might support wound healing process, due to its ability to modulate the expression of wound healing associated cytokines. These observations suggested that the APR-TD012 might be a potential therapeutic agent for HHD-lesions. View Full-Text
Keywords: Hailey–Hailey disease; oxidative-stress; keratinocytes Hailey–Hailey disease; oxidative-stress; keratinocytes
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Cialfi, S.; Calabro, S.; Franchitto, M.; Zonfrilli, A.; Screpanti, I.; Talora, C. Hypotonic, Acidic Oxidizing Solution Containing Hypochlorous Acid (HClO) as a Potential Treatment of Hailey-Hailey Disease. Molecules 2019, 24, 4427.

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