The invention relates to a novel substituted amino acids, agricultural compositions comprising the novel substituted amino acids, and their use for controlling undesired plant growth alone or in combination with crop protection agents such as pesticides or plant growth regulators.

The present invention relates to compounds of formula (I): Formula (I) wherein Q is selected from O or S; L is a saturated or unsaturated, optionally substituted C.sub.1-C.sub.12 hydrocarbylene group optionally including one or more heteroatoms N, O or S; R.sup.1 is —NR.sup.3R.sup.4, —OR.sup.5, —(C═NR.sup.6)R.sup.7, —(CO)R.sup.8, —CN, —N.sub.3, a quaternary ammonium group or an optionally substituted heterocycle; R.sup.3, R.sup.4, R.sup.5, R.sup.6, R.sup.7 and R.sup.8 are each independently hydrogen or a saturated or unsaturated, optionally substituted C.sub.1-C.sub.10 hydrocarbyl group optionally including one or more heteroatoms N, O or S; wherein optionally L and R.sup.3, or L and R.sup.4, or R.sup.3 and R.sup.4, or L and R.sup.5, or L and R.sup.6, or L and R.sup.7, or R.sup.6 and R.sup.7, or L and R.sup.8 together with the atom(s) to which they are attached may form a 3- to 12-membered, saturated or unsaturated, optionally substituted cyclic group; and R.sup.2 is a cyclic group substituted at the a-position, wherein R.sup.2 may optionally be further substituted; provided that the atom of L which is attached to the sulfur atom of the sulfonylurea group is a carbon atom and is not a ring atom of a heterocyclic or aromatic group. The present invention further relates to salts, solvates and prodrugs of such compounds, to pharmaceutical compositions comprising such compounds, and to the use of such compounds in the treatment and prevention of medical disorders and diseases, most especially by the inhibition of NLRP.sub.3. ##STR00001##

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-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, as 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.

Organic anolyte materials for redox flow batteries and redox flow batteries containing organic anolyte materials are disclosed.

Organic anolyte materials for redox flow batteries and redox flow batteries containing organic anolyte materials are disclosed.

The present invention relates to compounds of formula (I): Formula (I) wherein Q is selected from O or S; L is a saturated or unsaturated, optionally substituted C.sub.1-C.sub.12 hydrocarbylene group optionally including one or more heteroatoms N, O or S; R.sup.1 is NR.sup.3R.sup.4, OR.sup.5, (CNR.sup.6)R.sup.7, (CO)R.sup.8, CN, N.sub.3, a quaternary ammonium group or an optionally substituted heterocycle; R.sup.3, R.sup.4, R.sup.5, R.sup.6, R.sup.7 and R.sup.8 are each independently hydrogen or a saturated or unsaturated, optionally substituted C.sub.1-C.sub.10 hydrocarbyl group optionally including one or more heteroatoms N, O or S; wherein optionally L and R.sup.3, or L and R.sup.4, or R.sup.3 and R.sup.4, or L and R.sup.5, or L and R.sup.6, or L and R.sup.7, or R.sup.6 and R.sup.7, or L and R.sup.8 together with the atom(s) to which they are attached may form a 3- to 12-membered, saturated or unsaturated, optionally substituted cyclic group; and R.sup.2 is a cyclic group substituted at the a-position, wherein R.sup.2 may optionally be further substituted; provided that the atom of L which is attached to the sulfur atom of the sulfonylurea group is a carbon atom and is not a ring atom of a heterocyclic or aromatic group. The present invention further relates to salts, solvates and prodrugs of such compounds, to pharmaceutical compositions comprising such compounds, and to the use of such compounds in the treatment and prevention of medical disorders and diseases, most especially by the inhibition of NLRP.sub.3.

##STR00001##

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.

Organic anolyte materials for redox flow batteries and redox flow batteries containing organic anolyte materials are disclosed.

The present invention is directed towards compounds of formula (I), capable of binding to sortilin and thereby useful as sortilin inhibitors in the treatment of diseases, disorders and conditions associated with sortilin activity and binding of ligands, such as progranulin, to sortilin.

##STR00001##