The disclosure provides for new substituted cysteamine and cystamine compounds, pharmaceutical compositions made thereof, and methods thereof including the treatment of any disease or disorder in a subject that can benefit from one or more of the bioprotective effects of the compounds, including but not limited to, binding of cystine, reducing oxidative stress, increasing adiponectin levels and/or increasing brain-derived neurotrophic factors. Examples of such disease and disorders, include but are not limited to, cystinosis, and fatty liver diseases.

Provided is a novel production method for a fluorinated organic compound (1), the method comprising step A of fluorinating a hydrogen atom-containing organic compound (2) using IF.sub.5 in a liquid composition comprising an organic solvent, the amount of the hydrogen atom-containing organic compound (2) being 1.8 mol or more per liter of the organic solvent.

Provided is a novel production method for a fluorinated organic compound (1), the method comprising step A of fluorinating a hydrogen atom-containing organic compound (2) using IF.sub.5 in a liquid composition comprising an organic solvent, the amount of the hydrogen atom-containing organic compound (2) being 1.8 mol or more per liter of the organic solvent.

The present invention provides lipids that are advantageously used in lipid particles for the in vivo delivery of therapeutic agents to cells. In particular, the invention formula (I) provides lipids having the following structure XXXIII wherein: R.sub.1 and R.sub.2 are each independently for each occurrence optionally substituted C.sub.10-C.sub.30 alkyl, optionally substituted C.sub.10-C.sub.30 alkenyl, optionally substituted C.sub.10-C.sub.30 alkynyl, optionally substituted C.sub.10-C.sub.30 acyl, or -linker-ligand; R.sub.3 is H, optionally substituted C.sub.1-C.sub.10 alkyl, optionally substituted C.sub.2-C.sub.10 alkenyl, optionally substituted C.sub.2-C.sub.10 alkynyl, alkylhetrocycle, alkylphosphate, alkylphosphorothioate, alkylphosphorodithioate, alkylphosphonates, alkylamines, hydroxyalkyls, ω-aminoalkyls, ω-(substituted)aminoalkyls, ω-phosphoalkyls, ω-thiophosphoalkyls, optionally substituted polyethylene glycol (PEG, mw 100-40K), optionally substituted mPEG (mw 120-40K), heteroaryl, heterocycle, or linker-ligand; and E is C(O)O or OC(O). ##STR00001##

The present invention provides lipids that are advantageously used in lipid particles for the in vivo delivery of therapeutic agents to cells. In particular, the invention formula (I) provides lipids having the following structure XXXIII wherein: R.sub.1 and R.sub.2 are each independently for each occurrence optionally substituted C.sub.10-C.sub.30 alkyl, optionally substituted C.sub.10-C.sub.30 alkenyl, optionally substituted C.sub.10-C.sub.30 alkynyl, optionally substituted C.sub.10-C.sub.30 acyl, or -linker-ligand; R.sub.3 is H, optionally substituted C.sub.1-C.sub.10 alkyl, optionally substituted C.sub.2-C.sub.10 alkenyl, optionally substituted C.sub.2-C.sub.10 alkynyl, alkylhetrocycle, alkylphosphate, alkylphosphorothioate, alkylphosphorodithioate, alkylphosphonates, alkylamines, hydroxyalkyls, ω-aminoalkyls, ω-(substituted)aminoalkyls, ω-phosphoalkyls, ω-thiophosphoalkyls, optionally substituted polyethylene glycol (PEG, mw 100-40K), optionally substituted mPEG (mw 120-40K), heteroaryl, heterocycle, or linker-ligand; and E is C(O)O or OC(O). ##STR00001##

Described herein are compounds that are able to generate oct-2-en-4-one and thus to provide a long-lasting or substantive strawberry odor to an environment. Also described herein is a method of imparting a long-lasting strawberry odor to surfaces, such as hard surfaces, fabric, skin or hair. Also described herein is a method of using said compounds in perfumery. Also described herein are perfuming compositions or perfumed articles including the compounds.

Described herein are compounds that are able to generate oct-2-en-4-one and thus to provide a long-lasting or substantive strawberry odor to an environment. Also described herein is a method of imparting a long-lasting strawberry odor to surfaces, such as hard surfaces, fabric, skin or hair. Also described herein is a method of using said compounds in perfumery. Also described herein are perfuming compositions or perfumed articles including the compounds.

The invention features compositions, methods, and kits containing (i) one or more cysteamine precursor compounds convertible to cysteamine in vivo, and (ii) optionally agents to enhance that conversion, formulated to produce a spectrum of pharmacokinetic profiles of cysteamine that can be tailored to individual patients and diseases. The invention also features varying modes of administration of the therapeutic substances in the treatment of cystinosis and other cysteamine sensitive disorders. In particular, formulations combining active ingredient(s) with pharmaceutical excipients that permit sustained cysteamine plasma concentrations are featured.

The invention features compositions, methods, and kits containing (i) one or more cysteamine precursor compounds convertible to cysteamine in vivo, and (ii) optionally agents to enhance that conversion, formulated to produce a spectrum of pharmacokinetic profiles of cysteamine that can be tailored to individual patients and diseases. The invention also features varying modes of administration of the therapeutic substances in the treatment of cystinosis and other cysteamine sensitive disorders. In particular, formulations combining active ingredient(s) with pharmaceutical excipients that permit sustained cysteamine plasma concentrations are featured.

A compound represented by formula (i) has, as K.sup.i1 in the formula (i), a structure represented by any one of formula (K-1) to formula (K-3). When used in a liquid crystal composition, it adheres to substrates which hold the liquid crystal composition (liquid crystal layer) therebetween, thereby permitting liquid crystal molecules to be maintained in the state of being aligned in the vertical direction. The liquid crystal composition using the compound enables liquid crystal molecules to be aligned even when the PI layer is not provided (vertical alignment of liquid crystal molecules is induced without the voltage applied and horizontal alignment of liquid crystal molecules is realized with the voltage applied). It is possible to provide a polymerizable compound being excellent in storability and capable of uniform vertical alignment of liquid crystal molecules with no PI layer provided.