Derivatives of nifuroxazide for use in the treatment of mitochondrial disorders and neurodegenerative diseases

A method of treating diseases associated with mitochondrial dysfunction, comprising the step of administering to a patient in need thereof a therapeutically effective amount of a pharmaceutical composition comprising a compound Formula I:

• or a pharmaceutically acceptable salt or solvate thereof and at least one pharmaceutically acceptable excipient,
• wherein
• R1 and R2 are independently selected from H, C1-C6-alkyl, C3-C6-cycloalkyl and halogens, and
• Z is selected from
• wherein R3, R4, R5 and R6 are independently selected from H C1-C6-alkyl, C3-C6-cycloalkyl, OH and halogens.

A method for preventing mitochondrial dysfunction associated with CHCHD10 mutations, comprising the step of administering to a patient in need thereof a therapeutically effective amount of a compound of Formula I

• wherein
• R1 and R2 are independently selected from H, C1-C6-alkyl, C3-C6-cycloalkyl and halogens, and
• Z is selected from
• wherein R3, R4, R5 and R6 are independently selected from H, C1-C6-alkyl, C3-C6-cycloalkyl, OH and halogens,
• or a pharmaceutically acceptable salt or solvate thereof.

A compound of Formula I

• a pharmaceutically acceptable salt or a solvate thereof,
• wherein
• R1 and R2 are independently selected from H, C1-C6-alkyl, C3-C6-cycloalkyl and halogens, and
• Z is selected from
• wherein R3, R4, R5 and R6 are independently selected from H, C1-C6-alkyl, C3-C6-cycloalkyl, OH and halogens,
• with the proviso that R1 and R2 are not both H and that R3, R4, R5 and R6 are not all H.