116 results about "Mitochondrial Disorders" patented technology

Methods of treating or suppressing mitochondrial diseases, such as Friedreich's ataxia (FRDA), Leber's Hereditary Optic Neuropathy (LHON), mitochondrial myopathy, encephalopathy, lactacidosis, stroke (MELAS), or Kearns-Sayre Syndrome (KSS) are disclosed, as well as compounds useful in the methods of the invention. Energy biomarkers useful in assessing the metabolic state of a subject and the efficacy of treatment are also disclosed.

The present invention provides methods for rapid forensic analysis of mitochondrial DNA and methods for characterizing heteroplasmy of mitochondrial DNA, which can be used to assess the progression of mitochondrial diseases.

Disclosed herein are resorufin derivative compounds and methods of using such compounds for treating or suppressing oxidative stress disorders, including mitochondrial disorders, impaired energy processing disorders, neurodegenerative diseases and diseases of aging.

Abstract: The invention is based in part on the discovery that chromosomal disorders such as Down Syndrome can be diagnosed by assessing maternal mitochondrial status. Thus the invention relates to diagnostic and therapeutic methods and related products for chromosomal disorders such as Downs Syndrome, for example, for identifying a risk of fetal Downs syndrome and methods of mitigating that risk. The methods also re useful for other therapies where it is desirable to manipulate mitochondria such as tissue generation.

Disclosed herein are resorufin derivative compounds and methods of using such compounds for treating or suppressing oxidative stress disorders, including mitochondrial disorders, impaired energy processing disorders, neurodegenerative diseases and diseases of aging.

The present invention relates to a pharmaceutical or veterinary or nutritional or personal care composition comprising coenzyme Q10 (CoQ10), reduced CoQ10, or mixtures thereof and oxygenated dibenzo-α-pyrone or an amino acyl ester thereof. The composition of the present invention is able to support and / or provide therapy to individuals at risk and / or under treatment for dysfunctions of energy metabolism, and specifically, for mitochondrial diseases.

The invention is based in part on the discovery that chromosomal disorders such as Down Syndrome can be diagnosed by assessing maternal mitochondrial status. Thus the invention relates to diagnostic and therapeutic methods and related products for chromosomal disorders such as Downs Syndrome, for example, for identifying a risk of fetal Downs Syndrome and methods of mitigating that risk. The methods also are useful for other therapies where it is desirable to manipulate mitochondria such as tissue generation.

A novel substituted N-acetyl-L-cysteine (NAC) derivative and methods of using this compound for the treatment of diseases and / or conditions, including but not limited to diseases and / or conditions of, or involving, the Central Nervous System (CNS), including schizophrenia adrenoleukodystrophy, mitochondrial diseases (e.g. Leigh syndrome, Alpers' disease, and MELAS), Huntington's disease, trichotillomania, HIV-associated neurocognitive disorder, hypoxic-ischemic encephalopathy, drug craving, and drug addiction.

Disclosed herein are compounds and methods of using such compounds for treating or suppressing oxidative stress disorders, including mitochondrial disorders, impaired energy processing disorders, neurodegenerative diseases and diseases of aging, or for modulating one or more energy biomarkers, normalizing one or more energy biomarkers, or enhancing one or more energy biomarkers, wherein the compounds are tocopherol quinone derivatives. Further disclosed are compounds, compositions, and methods for treatment of, or prophylaxis against, radiation exposure.

Disclosed herein are compounds and methods of using such compounds for treating or suppressing oxidative stress disorders, including mitochondrial disorders, impaired energy processing disorders, neurodegenerative diseases and diseases of aging, or for modulating one or more energy biomarkers, normalizing one or more energy biomarkers, or enhancing one or more energy biomarkers, wherein the compound is a compound of Formula I or Formula II: wherein: R1 and R2 are independently hydrogen, (C1-C6)alkyl or —O(C1-C6)alkyl; or R1 and R2 together represent —CH═CH—CH═CH—; R3 is (C1-C6)alkyl; X is —CH═CH— or —C≡C—; m is 1-10; n is 1-5; k is 1-3, with the proviso that when k is an integer of 2 or 3, n is independently 1-5 in each occurrence of the —X—(CH2)n— group; Y is —OR4, —CN, —C(═O)OR5, —C(═O)R5, or —C(═O)N(R6)2; R4 and R5 are independently selected from the group consisting of hydrogen, —(C1-C6)alkyl, —(C1-C6)haloalkyl, —C(═O)—(C1-C6)alkyl, —C(═O)—(C1-C6)haloalkyl, —C(═O)—NH(C1-C6)alkyl, —C(═O)—N((C1-C6)alkyl)2, —C(═O)—NH2, and phenyl, wherein the phenyl group may optionally be substituted with a substituent selected from the group consisting of —(C1-C6)alkyl, —O—(C1-C6)alkyl, —(C1-C6)haloalkyl, and halo; each R6 is independently hydrogen or —(C1-C6)alkyl; and M is —H, —C(O)—R7 or —C(O)O—R7, wherein R7 is —(C1-C6)alkyl or phenyl; or a stereoisomer, mixture of stereoisomers, pharmaceutically acceptable salt, crystalline form, non-crystalline form, hydrate or solvate thereof.

Disclosed are novel fusion protein constructs comprising a functional mitochondrial protein, that can enter mitochondria within intact cells. Further disclosed are methods of treating mitochondrial disorders by the disclosed fusion proteins and compositions therefor.

Novel substituted N-acetyl-L-cysteine (NAC) derivatives and related compounds and methods of using these compounds for the treatment of diseases and / or conditions, including but not limited to diseases and / or conditions of, or involving, the Central Nervous System (CNS), including schizophrenia adrenoleukodystrophy, mitochondrial diseases (e.g. Leigh syndrome, Alpers' disease, and MELAS), Huntington's disease, trichotillomania, HIV-associated neurocognitive disorder, hypoxic-ischemic encephalopathy, drug craving, and drug addiction.

The invention relates to a soft capsule for treating nerve diseases. The soft capsule comprises a liquid medicine and a soft capsule casing for sealing the liquid medicine, wherein the liquid medicine contains active ingredients. The medicine composition of the soft capsule is the only medicine which achieves double improvements on brain metabolism and psychiatric symptoms and has no direct competitor at present; and the soft capsule is a brain metabolism improver and cranial nerve cell protective agent, can prevent mitochondrial diseases, inhibit platelet aggregation and improve microcirculation effectively, has an effective antioxidation function, and is also a preferred medicine for treating FRDA (Friedreich ataxia). The successful development of the soft capsule can provide enormous economic and social benefits for the pharmacy industry.