Methods of treating or suppressing mitochondrial diseases, such as Friedreich's ataxia (FRDA), Leber's Hereditary Optic Neuropathy (LHON), mitochondrial myopathy, encephalopathy, lactacidosis, and stroke (MELAS), Kearns-Sayre Syndrome (KSS), are disclosed, as well as compounds useful in the methods of the invention, such as 4-(p-quinoly)-2-hydroxybutanamide derivatives. Methods and compounds useful in treating other disorders such as amyotrophic lateral sclerosis (ALS), Huntington's disease, Parkinson's disease, and pervasive developmental disorders such as autism are also disclosed. Energy biomarkers useful in assessing the metabolic state of a subject and the efficacy of treatment are also disclosed. Methods of modulating, normalizing, or enhancing energy biomarkers, as well as compounds useful for such methods, are also disclosed.

Methods of treating or suppressing mitochondrial diseases, such as Friedreich's ataxia (FRDA), Leber's Hereditary Optic Neuropathy (LHON), mitochondrial myopathy, encephalopathy, lactacidosis, and stroke (MELAS), Kearns-Sayre Syndrome (KSS), are disclosed, as well as compounds useful in the methods of the invention, such as 4-(p-quinoly)-2-hydroxybutanamide derivatives. Methods and compounds useful in treating other disorders such as amyotrophic lateral sclerosis (ALS), Huntington's disease, Parkinson's disease, and pervasive developmental disorders such as autism are also disclosed. Energy biomarkers useful in assessing the metabolic state of a subject and the efficacy of treatment are also disclosed. Methods of modulating, normalizing, or enhancing energy biomarkers, as well as compounds useful for such methods, are also disclosed.

Provided herein are compounds, pharmaceutical compositions, and methods of use thereof, including methods of treating neurological disorders. For example, provided herein is a compound of Formula I:

##STR00001##

or a pharmaceutically acceptable salt thereof, wherein values for the variables (e.g., X.sup.1, R.sup.1A, R.sup.1B, R.sup.2A, R.sup.2B, R.sup.3A, R.sup.3B, R.sup.3C, R.sup.3D) are as disclosed herein. The compounds disclosed herein (e.g., compounds of Formula I, or pharmaceutically acceptable salts thereof) and pharmaceutical compositions can be used to treat neurological disorders.

Provided herein are compounds, pharmaceutical compositions, and methods of use thereof, including methods of treating neurological disorders. For example, provided herein is a compound of Formula I:

##STR00001##

or a pharmaceutically acceptable salt thereof, wherein values for the variables (e.g., X.sup.1, R.sup.1A, R.sup.1B, R.sup.2A, R.sup.2B, R.sup.3A, R.sup.3B, R.sup.3C, R.sup.3D) are as disclosed herein. The compounds disclosed herein (e.g., compounds of Formula I, or pharmaceutically acceptable salts thereof) and pharmaceutical compositions can be used to treat neurological disorders.

This invention is related to a compound with the structural formula (I)

##STR00001## wherein, R1, and R2 are independently selected from the group consisting of C.sub.1-C.sub.6 alkyl and is preferably methyl, ethyl, propyl or isopropyl; R3 is selected from the group consisting of CH.sub.2NHR.sub.9, C(?O)YR.sub.10, CH2OH

##STR00002##

This invention is related to a compound with the structural formula (I)

##STR00001## wherein, R1, and R2 are independently selected from the group consisting of C.sub.1-C.sub.6 alkyl and is preferably methyl, ethyl, propyl or isopropyl; R3 is selected from the group consisting of CH.sub.2NHR.sub.9, C(?O)YR.sub.10, CH2OH

##STR00002##

Solid state forms of Abexinostat salts and of Abexinostat tosylate:p-toluenesulfonic acid monohydrate complex, processes for preparation thereof, pharmaceutical compositions and uses thereof are disclosed.

The invention relates to novel compounds that are useful for modulating cellular ROS. The compounds are amide-derivatives of 2-hydroxy-2-methyl-4-(3,5,6-trimethyl-1,4-benzoquinon-2-yl)-butanoic acid. The compounds of the invention are formulated into pharmaceutical or cosmetic compositions. The invention further relates to methods wherein the compounds of the invention are used for treating or preventing diseases associated with increased ROS levels mitochondrial disorders and/or conditions associated with mitochondrial dysfunction, including adverse drug effects. The invention also relates to cosmetic methods for treating or delaying further aging of the skin and veterinary applications.

The invention relates to novel compounds that are useful for modulating cellular ROS. The compounds are amide-derivatives of 2-hydroxy-2-methyl-4-(3,5,6-trimethyl-1,4-benzoquinon-2-yl)-butanoic acid. The compounds of the invention are formulated into pharmaceutical or cosmetic compositions. The invention further relates to methods wherein the compounds of the invention are used for treating or preventing diseases associated with increased ROS levels mitochondrial disorders and/or conditions associated with mitochondrial dysfunction, including adverse drug effects. The invention also relates to cosmetic methods for treating or delaying further aging of the skin and veterinary applications.

The invention relates to methods, compositions, devices, systems and kits as described including, without limitation, reagents and mixtures for determining the identity of nucleic acids in nucleotide sequences using, for example, sequencing by synthesis methods. In particular, the present invention contemplates the use of photoprotective mixture of compounds as imaging reagents to improve stability and storage of fluorescent compounds, including but not limited to, nucleotides with fluorescent labels.