Compositions comprising amino acids for use in the treatment of mitochondrial dysfunction-related diseases

1. A method of treatment for a mitochondrial dysfunction-related neurodegenerative disease, comprising administering to a subject in need thereof a therapeutically effective amount of a composition comprising an active agent, said active agent comprising leucine, isoleucine, valine, threonine, lysine, citric acid, succinic acid and malic acid,

wherein the citric acid:malic acid:succinic acid weight ratio is between 10:1:1 and 1.3:1:1,

wherein the composition comprises the amino acids isoleucine, leucine and valine in an amount by weight between 35% and 65%,

wherein the weight ratio between (i) the sum of citric acid, malic acid and succinic acid and (ii) the sum of leucine, isoleucine and valine is between 0.1 and 0.4,

wherein the weight ratio between (i) the sum of citric acid, malic acid and succinic acid and (ii) the sum of leucine, isoleucine, valine, lysine and threonine is between 0.05 and 0.3, and

wherein the weight ratio between leucine and citric acid is comprised between 5 and 1, wherein the mitochondrial dysfunction-related neurodegenerative disease is selected in the group consisting of Alzheimer's disease, Parkinson's disease, and Huntington's disease.

2. The method of claim 1 , wherein the weight ratio between citric acid and the sum of malic acid and succinic acid is between 1.0 and 4.0.

3. The method of claim 1 , wherein the weight ratio between citric acid and the sum of malic acid and succinic acid is between 1.5 and 2.5.

4. The method of claim 1 , wherein the citric acid:malic acid:succinic acid weight ratio is between 10:1:1 and 2:1.5:1.5.

5. The method of claim 1 , wherein the citric acid:malic acid:succinic acid weight ratio is between 7:1:1 and 1.5:1:1.

6. The method of claim 1 , wherein the citric acid:malic acid:succinic acid weight ratio is between 5:1:1 and 3:1:1.

7. The method of claim 1 , wherein said active agent further comprises at least one amino acid selected from the group consisting of histidine, phenylalanine, methionine, tryptophan, tyrosine, and cysteine.

8. The method of claim 1 , wherein said active agent further comprises histidine, phenylalanine, methionine, tryptophan and cysteine.

9. The method of claim 1 , wherein said active agent further comprises histidine, phenylalanine, methionine, tryptophan, cysteine and tyrosine.

10. The method of claim 8 , wherein the ratio between the overall molar amount of citric acid, malic acid and succinic acid, and the overall molar amount of methionine, phenylalanine, histidine and tryptophan is higher than 1.35.

11. The method of claim 1 , wherein the ratio between the overall molar amount of citric acid, succinic acid and malic acid, and the overall molar amount of lysine and threonine is between 0.1 and 0.7.

12. The method of claim 1 , wherein the ratio between the overall molar amount of citric acid, succinic acid and malic acid, and the overall molar amount of lysine and threonine is between 0.15 and 0.55.

13. The method of claim 1 , wherein the weight or molar amount of citric acid is higher than the overall weight or molar amount of malic acid and succinic acid.

14. The method of claim 1 , wherein the weight ratio between leucine and citric acid is between 2.50 and 3.50.

15. The method of claim 1 , wherein said active agent is free of arginine.

16. The method of claim 1 , wherein the composition further comprises one or more vitamins.

17. The method of claim 1 , wherein the composition further comprises one or more vitamins selected from the group of vitamins B.