IMPAIRED ENERGY PROCESSING DISORDERS AND MITOCHONDRIAL DEFICIENCY
Some aspects of the invention provide for a method of treating Impaired Energy Processing Disorders and Mitochondrial Deficiencies using polyunsaturated fatty acids which are modified in certain positions to attenuate oxidative damage by Reactive Oxygen Species (ROS) and/or suppress the rate of formation of reactive products and toxic compounds.
1 .- 15 . (canceled)
16 . A method of treating or inhibiting progression of impaired energy processing disorder or mitochondrial deficiency, comprising:
repeatedly administering an effective amount of a deuterated polyunsaturated fatty acid or fatty acid ester to a patient;
wherein the amount of deuterated polyunsaturated fatty acid or fatty acid ester administered to or ingested by the patient is in the range of about 1% to about 99% of the total amount of polyunsaturated fatty acid or fatty acid ester administered to or ingested by the patient; and
wherein the deuterated polyunsaturated fatty acid or fatty acid ester is arachidonic acid, an arachidonic acid ester, eicosapentaenoic acid, an eicosapentaenoic acid ester, docosahexaenoic acid, a docosahexaenoic acid ester, linoleic acid, a linoleic acid ester, linolenic acid, or a linolenic acid ester.
17 . The method of claim 16 , wherein the impaired energy processing disorder or mitochondrial deficiency is selected from: Co-enzyme Q deficiency; Diabetes mellitus and deafness (DAD); Maternally Inherited Diabetes and Deafness (MIDD); Friedreich's ataxia (FA); Leber's congenital amaurosis; Leber's hereditary optic neuropathy (LHON); Leigh syndrome; Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke (MELAS) syndrome; Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE); Myoclonus Epilepsy Associated with Ragged-Red Fibers (MERRF) syndrome; Myoneurogenetic gastrointestinal encephalopathy (MNGIE) and neuropathy; Neuropathy, ataxia, retinitis pigmentosa, and ptosis (NARP); Wolff-Parkinson-White syndrome; X-linked Adrenoleukodystrophy (X-ALD), Fanconi's syndrome and Pearson's syndrome.
18 . The method of claim 17 , wherein the impaired energy processing disorder or mitochondrial deficiency is Friedreich's ataxia.
19 . The method of claim 16 , wherein the deuterated polyunsaturated fatty acid or fatty acid ester is deuterated at a level greater than 0.02%.
20 . The method of claim 16 , wherein the deuterated polyunsaturated fatty acid or fatty acid ester administered to or ingested by the patient comprises between about 1% and 50% of the total amount of fatty acids or fatty acid esters administered to or ingested by the patient.
21 . The method of claim 16 , wherein the deuterated polyunsaturated fatty acid or fatty acid ester administered to or ingested by the patient comprises between about 1% and 30% of the total amount of fatty acids or fatty acid esters administered to or ingested by the patient.
22 . The method of claim 16 , wherein the deuterated polyunsaturated fatty acid or fatty acid ester administered to or ingested by the patient comprises between about 20% and 50% of the total amount of fatty acids or fatty acid esters administered to or ingested by the patient.
23 . The method of claim 16 , wherein the deuterated fatty acid or fatty acid ester is arachidonic acid.
24 . The method of claim 16 , wherein the deuterated fatty acid or fatty acid ester is an arachidonic acid ester.
25 . The method of claim 16 , wherein the deuterated fatty acid or fatty acid ester is docosahexaenoic acid.
26 . The method of claim 16 , wherein the deuterated fatty acid or fatty acid ester is a docosahexaenoic acid ester.
27 . The method of claim 16 , wherein the deuterated fatty acid or fatty acid ester is linoleic acid.
28 . The method of claim 16 , wherein the deuterated fatty acid or fatty acid ester is a linoleic acid ester.
29 . The method of claim 16 , wherein the deuterated fatty acid or fatty acid ester is linolenic acid.
30 . The method of claim 16 , wherein the deuterated fatty acid or fatty acid ester is a linolenic acid ester.
31 . The method of claim 16 , wherein the deuterated polyunsaturated fatty acid or fatty acid ester is selected from the group consisting of 11,11-D2-linolenic acid, 14,14-D2-linolenic acid, 11,11,14,14-D4-linolenic acid, 11,11-D2-linoleic acid, 11-D-linolenic acid, 14-D-linolenic acid, 11,14-D2-linolenic acid, and 11-D-linoleic acid.
32 . The method of claim 16 , wherein the deuterated polyunsaturated fatty acid or fatty acid ester is selected from the group consisting of a 11,11-D2-linolenic acid ester, a 14,14-D2-linolenic acid ester, a 11,11,14,14-D4-linolenic acid ester, a 11,11-D2-linoleic acid ester, a 11-D-linolenic acid ester, a 14-D-linolenic acid ester, a 11,14-D2-linolenic acid ester, and a 11-D-linoleic acid ester.
33 . The method of claim 16 , wherein the ester is a triglyceride, diglyceride, or monoglyceride.
34 . The method of claim 16 , further comprising co-administering an antioxidant.
35 . The method of claim 34 , wherein the antioxidant is idebenone, mitoquinone, mitoquinol, vitamin C, or vitamin E.
36 . The method of claim 16 , wherein the amount of deuterated polyunsaturated fatty acid or fatty acid ester administered to or ingested by the patient is in the range of about 0.1 mg/kg to 100 mg/kg per day.
37 . The method of claim 16 , wherein the deuterated polyunsaturated fatty acid or fatty acid ester is 11,11-D2-linoleic acid.
38 . The method of claim 16 , wherein the deuterated polyunsaturated fatty acid or fatty acid ester is 11,11-D2-linoleic acid ethyl ester.
39 . The method of claim 16 , wherein the deuterated polyunsaturated fatty acid or fatty acid ester is 11,11,14,14-D4-linolenic acid.
40 . The method of claim 16 , wherein the deuterated polyunsaturated fatty acid or fatty acid ester is 11,11,14,14-D4-linolenic acid ethyl ester.