In one aspect, compounds of Formula AA, or a pharmaceutically acceptable salt thereof, are featured. The variables shown in Formula AA are as defined in the claims. The compounds of formula AA are NLRP3 activity modulators and, as such, can be used in the treatment of metabolic disorders (e.g. Type 2 diabetes, atherosclerosis, obesity or gout), a disease of the central nervous system (e.g. Alzheimer's disease, multiple sclerosis, Amyotrophic Lateral Sclerosis or Parkinson's disease), lung disease (e.g. asthma, COPD or pulmonary idiopathic fibrosis), liver disease (e.g. NASH syndrome, viral hepatitis or cirrhosis), pancreatic disease (e.g. acute pancreatitis or chronic pancreatitis), kidney disease (e.g. acute kidney injury or chronic kidney injury), intestinal disease (e.g. Crohn's disease or Ulcerative Colitis), skin disease (e.g. psoriasis), musculoskeletal disease (e.g. scleroderma), a vessel disorder (e.g. giant cell arteritis), a disorder of the bones (e.g. osteoarthritis, osteoporosis or osteopetrosis disorders), eye disease (e.g. glaucoma or macular degeneration), a disease caused by viral infection (e.g. HIV or AIDS), an autoimmune disease (e.g. Rheumatoid Arthritis, Systemic Lupus Erythematosus or Autoimmune Thyroiditis), cancer or aging.

##STR00001##

Certain embodiments are directed hydrophilic redox substrates for leukocyte esterase (LE). In certain aspects a hydrophilic redox leukocyte esterase substrate or a small molecule enzyme substrate for use in methods and/or devices to electrochemically detect and/or measure LE activity in a sample.

Certain embodiments are directed hydrophilic redox substrates for leukocyte esterase (LE). In certain aspects a hydrophilic redox leukocyte esterase substrate or a small molecule enzyme substrate for use in methods and/or devices to electrochemically detect and/or measure LE activity in a sample.

This invention describes a method of conjugating a cell binding agent such as an antibody with an effector group (e.g., a cytotoxic agent) or a reporter group (e.g., a radionuclide), whereby the reporter or effector group is first reacted with a bifunctional linker and the mixture is then used without purification for the conjugation reaction with the cell binding agent. The method described in this invention is advantageous for preparation of stably-linked conjugates of cell binding agents, such as antibodies with effector or reporter groups. This conjugation method provides in high yields conjugates of high purity and homogeneity that are without inter-chain cross-linking and inactivated linker residues.

This invention describes a method of conjugating a cell binding agent such as an antibody with an effector group (e.g., a cytotoxic agent) or a reporter group (e.g., a radionuclide), whereby the reporter or effector group is first reacted with a bifunctional linker and the mixture is then used without purification for the conjugation reaction with the cell binding agent. The method described in this invention is advantageous for preparation of stably-linked conjugates of cell binding agents, such as antibodies with effector or reporter groups. This conjugation method provides in high yields conjugates of high purity and homogeneity that are without inter-chain cross-linking and inactivated linker residues.

A process for preparing a polyfluorinated compound of formula Ar—R.sub.1 (I), wherein Ar—R.sub.1 (I) is an aromatic ring system

##STR00001##

wherein R.sub.1 is selected from the group consisting of SF.sub.4Cl, SF.sub.3, SF.sub.2CF.sub.3, TeF.sub.5, TeF.sub.4CF.sub.3, SeF.sub.3, IF.sub.2, SeF.sub.2CF.sub.3, and IF.sub.4, X.sub.2 is N or CR.sub.2, X.sub.3 is N or CR.sub.3, X.sub.4 is N or CR.sub.4, X.sub.5 is N or CR.sub.5, X.sub.6 is N or CR.sub.6, and the total number of nitrogen atoms in the aromatic ring system is between 0 and 3, wherein R.sub.2, R.sub.3, R.sub.4, R.sub.5 and R.sub.6 are independently selected from the group consisting of hydrogen, fluoro, chloro, bromo, nitro, trifluoromethyl, 2,2,2-trifluoroethyl, pentafluorosulfanyl, phthalimido, azido, benzyloxy, trifluoromethoxy, 2,2,2-trifluoroethoxy, methoxycarbonyl, ethoxycarbonyl, methylcarbonyl, ethylcarbonyl, acetoxy, t-butyl, phenylcarbonyl, benzylcarbonyl, 3-trifluoromethylphenyl, phenylsulfonyl, methylsulfonyl, chlorophenyl, methyldoxolonyl, methyl, isopropyl, cyclopropyl, cyclobutyl, cyclopentyl, cyclohexyl, fluoromethyl, fluoroethyl and phenyl.

The invention discloses compounds of Formula (I), ##STR00001##
wherein A.sup.1, R.sup.1, R.sup.2, R.sup.3, R.sup.4, and n are as defined herein. The present invention relates to compounds and their use in the treatment of cystic fibrosis, methods for their production, pharmaceutical compositions comprising the same, and methods of treating cystic fibrosis by administering a compound of the invention.

The invention discloses compounds of Formula (I), ##STR00001##
wherein A.sup.1, R.sup.1, R.sup.2, R.sup.3, R.sup.4, and n are as defined herein. The present invention relates to compounds and their use in the treatment of cystic fibrosis, methods for their production, pharmaceutical compositions comprising the same, and methods of treating cystic fibrosis by administering a compound of the invention.

The invention discloses compounds of Formula (I), ##STR00001##
wherein A.sup.1, R.sup.1, R.sup.2, R.sup.3, R.sup.4, and n are as defined herein. The present invention relates to compounds and their use in the treatment of cystic fibrosis, methods for their production, pharmaceutical compositions comprising the same, and methods of treating cystic fibrosis by administering a compound of the invention.

The invention discloses compounds of Formula (I), ##STR00001##
wherein A.sup.1, R.sup.1, R.sup.2, R.sup.3, R.sup.4, and n are as defined herein. The present invention relates to compounds and their use in the treatment of cystic fibrosis, methods for their production, pharmaceutical compositions comprising the same, and methods of treating cystic fibrosis by administering a compound of the invention.