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, an well as compounds useful in the methods of the invention, such as 4-(p-quinolyl)-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, an well as compounds useful in the methods of the invention, such as 4-(p-quinolyl)-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.

The invention relates to plasminogen activator-1 (PAI-1) inhibitor compounds and uses thereof in the treatment of any disease or disorder associated with elevated PAI-1. The invention includes, but is not limited to, the use of such compounds to prevent or reduce thrombosis and fibrosis, to promote thrombolysis, and to modulate lipid metabolism and treat diseases or disorders associated with elevated PAI-1, cholesterol, or lipid levels.

The invention relates to plasminogen activator-1 (PAI-1) inhibitor compounds and uses thereof in the treatment of any disease or disorder associated with elevated PAI-1. The invention includes, but is not limited to, the use of such compounds to prevent or reduce thrombosis and fibrosis, to promote thrombolysis, and to modulate lipid metabolism and treat diseases or disorders associated with elevated PAI-1, cholesterol, or lipid levels.

The present invention relates to a pharmaceutical composition and a cosmetic composition, which contain, as an active ingredient, a novel pseudo compound having similar structural characteristics to natural ceramide 1, or a pharmaceutically acceptable salt or solvate thereof, and the composition containing the compound of the present invention as an active ingredient has an excellent effect of enhancing a skin barrier function, thereby exhibiting an effect of alleviating inflammatory skin diseases, and has an excellent skin barrier improving function and an excellent skin moisturization enhancing effect.

The present invention relates to a pharmaceutical composition and a cosmetic composition, which contain, as an active ingredient, a novel pseudo compound having similar structural characteristics to natural ceramide 1, or a pharmaceutically acceptable salt or solvate thereof, and the composition containing the compound of the present invention as an active ingredient has an excellent effect of enhancing a skin barrier function, thereby exhibiting an effect of alleviating inflammatory skin diseases, and has an excellent skin barrier improving function and an excellent skin moisturization enhancing effect.

In one aspect, the invention relates to compounds having the formula: ##STR00001##
where R.sup.1-R.sup.5 and X are as defined in the specification, or a pharmaceutically acceptable salt thereof. These compounds have neprilysin inhibition activity. In another aspect, the invention relates to pharmaceutical compositions comprising such compounds; methods of using such compounds; and processes and intermediates for preparing such compounds.

In one aspect, the invention relates to compounds having the formula: ##STR00001##
where R.sup.1-R.sup.5 and X are as defined in the specification, or a pharmaceutically acceptable salt thereof. These compounds have neprilysin inhibition activity. In another aspect, the invention relates to pharmaceutical compositions comprising such compounds; methods of using such compounds; and processes and intermediates for preparing such compounds.

This document discloses molecules having the following formula (Formula One):

##STR00001##

and processes associated therewith.

This document discloses molecules having the following formula (Formula One):

##STR00001##

and processes associated therewith.