Compounds for altering levels of one or more nka alpha subunits and their use in treating prion diseases or brain diseases associated with cellular prion protein

A method for treating and/or preventing prion diseases and/or brain diseases, disorders or conditions that benefit from reduced levels of the cellular prion protein (PrPC) and/or altered levels of NKA alpha subunits comprising administering an effective amount of one or more agents that alter levels of one or more NKA alpha subunits, in a subject in need thereof, wherein each NKA alpha subunit with altered levels is a different paralog, and

• wherein the one or more agents that alter levels of one or more NKA alpha subunits are one or more compounds of Formula I, and/or a pharmaceutically acceptable salt and/or solvate thereof:
• wherein
• R1 is selected from C1-4alkyl and C1-4fluoroalkyl;
• R2 and R3 are independently selected from H, OC1-4alkyl and OC1-4fluoroalkyl;
• and
• X is selected from ═O, ═NH, NH2, NHC1-4alkyl, and N(C1-4alkyl)2.

A compound of Formula I-A or a pharmaceutically acceptable salt and/or solvate thereof:

• wherein
• R1 is selected from C1-4alkyl and C1-4fluoroalkyl;
• R2 and R3 are independently selected from H, OC1-4alkyl and OC1-4fluoroalkyl;
• and
• X is selected from ═O, ═NH and NH2;
• provided when X is ═O, R2 is H and R3 is OCH3, then R1 is not CH3 or CF3;
• and
• provided when X is NH2, R2 is H and R3 is OCH3, then R1 is not CH3.