Use of isothiocyanate functional surfactants as NRF2 inducers to treat epidermolysis bullosa simplex and related diseases
The present invention relates to methods and compositions for the prevention and treatment of keratin-based skin diseases. In particular, the application describes compositions and methods of treating a patient suffering from skin blistering comprising the use of phase II enzyme inducers and/or activity modifiers.
1. A method for treating a patient having dowling-meara type epidermolysis bullosa simplex, comprising the step of administering to the patient an isothiocyanate functional surfactant, wherein the protonated form of the isothiocyanate functional surfactant is represented by the following chemical structure:
wherein R 1 is selected from the group consisting of an alkyl, aryl, and alkenyl group containing 1 to 25 carbon atom(s); wherein R 2 is selected from the group consisting of NCS; and wherein R 3 -R 5 are each independently selected from the group consisting of H; OH; and an alkyl, aryl, alkaryl, aralkyl, alkoxy, alkanoyl, aroyl, and alkenyl group containing 1 to 25 carbon atom(s) with the proviso that at least one of R 3 -R 5 is selected from the group consisting of an alkyl, aryl, alkaryl, aralkyl, alkoxy, alkanoyl, aroyl, and alkenyl, group containing 8 to 25 carbon atoms.
2. The method according to claim 1 , wherein the protonated form of the isothiocyanate functional surfactant is represented by the following chemical structure:
wherein X is an integer ranging from 1 to 25, and wherein Y is an integer ranging from 6 to 25.
3. The method according to claim 1 , wherein the protonated form of the isothiocyanate functional surfactant is represented by the following chemical structure: