METHODS FOR THE TREATMENT OF MITOCHONDRIAL DISEASE
The disclosure provides methods of preventing, ameliorating or treating disruption of mitochondrial function and symptoms thereof. The methods provide administering aromatic-cationic peptides in effective amounts to prevent, treat or ameliorate the disruption of mitochondrial oxidative phosphorylation in a cell such as that found in a subject suffering from, or predisposed to a mitochondrial disease or disorder. In some embodiments, the methods comprise administering to a subject suffering from, or at risk for a mitochondrial disease or disorder, an effective amount of an aromatic-cationic peptide to subjects in need thereof.
1 .- 20 . (canceled)
21 . A method for treating mitochondrial disease resulting from nuclear DNA mutations in a subject in need thereof, the method comprising: administering to the subject a therapeutically effective amount of peptide D-Arg-2′,6′-Dmt-Lys-Phe-NH 2 or a pharmaceutically acceptable salt thereof.
22 . The method of claim 21 , wherein the nuclear DNA mutations result in mitochondrial DNA (mtDNA) depletion in the subject.
23 . The method of claim 21 , wherein the subject suffers from mitochondrial DNA (mtDNA) depletion syndrome.
24 . The method of claim 22 , wherein the subject suffers from mitochondrial DNA (mtDNA) depletion syndrome.
25 . The method of claim 21 , wherein the mitochondrial disease results from a mutation in the POLG gene.
26 . The method of claim 22 , wherein the mitochondrial disease results from a mutation in the POLG gene.
27 . The method of claim 23 , wherein the mitochondrial disease results from a mutation in the POLG gene.
28 . The method of claim 21 , wherein the mitochondrial disease results from a mutation in the SURF1 gene.
29 . The method of claim 22 , wherein the mitochondrial disease results from a mutation in the SURF1 gene.
30 . The method of claim 23 , wherein the mitochondrial disease results from a mutation in the SURF1 gene.
31 . A method for treating a subject suffering from mitochondrial DNA (mtDNA) depletion syndrome, the method comprising: administering to the subject a therapeutically effective amount of peptide D-Arg-2′,6′-Dmt-Lys-Phe-NH 2 or a pharmaceutically acceptable salt thereof.
32 . The method of claim 31 , wherein the subject has a mutation in the POLG gene.
33 . The method of claim 31 , wherein the subject has a mutation in the SURF1 gene.