Disclosed are an S-(carboxymethyl)-cysteine pharmaceutical compound (I), and a preparation method and use thereof. Also disclosed are an S-(carboxymethyl)-D-cysteine ammonium salt monohydrate, and use thereof in preparation of medicines for preventing and treating respiratory system diseases such as chronic obstructive pulmonary diseases, in particular in preparation of expectorants. The compounds can reduce airway resistance and production of oxides in rat COPD models, increase the level of antioxidants, and alleviate damage caused by the oxides and inflammatory mediators to lungs.

A method for producing P.sup.1,P.sup.4-di(uridine 5-)tetraphosphate (UP.sub.4U) that can avoid reduction of the synthetic efficiency without using UTP free is developed.

A method for producing UP.sub.4U comprising reacting a phosphoric acid-activating compound represented by formula [II] or [III] with a phosphoric acid compound selected from the group consisting of UMP, UDP, UTP and a pyrophosphoric acid or a salt thereof (excluding UTP free) in water or a hydrophilic organic solvent, in the presence of a metal ion selected from the group consisting of an iron (II) ion, an iron (III) ion, a trivalent aluminum ion, a trivalent lanthanum ion, and a trivalent cerium ion.

where, in the formula [II], R.sup.1 represents a uridyl group binding to the 5-position, X represents a heterocyclic group, and n represents an integer of 1 or 2,
where, in the formula [III], X represents a heterocyclic group selected from the group consisting of an imidazolyl group, a benzimidazolyl group, and a 1,2,4-triazolyl group.

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Provided are pharmaceutical composition particles which are capable of achieving both of masking of an unpalatable taste and improvement in dissolution properties; an orally disintegrating tablet including the pharmaceutical composition particles; and a method for manufacturing the pharmaceutical composition particles. Each of the pharmaceutical composition particles includes: a core particle containing a water-soluble gelling swelling substance; an intermediate layer containing a drug and coating an outside of the core particle; and an outer layer containing a water-insoluble substance and coating an outside of the intermediate layer. In addition, the orally disintegrating tablet includes the above-described pharmaceutical composition particles.

Provided herein are complexes comprising amphotericin B (AmB) or derivatives and sterols. Also provided herein are methods of treating cystic fibrosis using AmB or complexes comprising AmB or derivatives and sterols.

Provided herein are complexes comprising amphotericin B (AmB) or derivatives and sterols. Also provided herein are methods of treating cystic fibrosis using AmB or complexes comprising AmB or derivatives and sterols.

The present invention relates to a novel siRNA, and a high-efficiency double-stranded oligo RNA structure containing the same, and a nanoparticle containing the high-efficiency double-stranded oligo RNA structure. The double-stranded oligo RNA structure has a structure in which a hydrophilic material and a hydrophobic material are conjugated to both ends of a double-stranded oligo RNA (siRNA) via a simple covalent bond or linker-mediated covalent bond in order to be efficiently delivered into cells, and may be converted into a nanoparticle form in an aqueous solution by hydrophobic interactions of double-stranded oligo RNA structures. It is preferable that the siRNA contained in the double-stranded oligo RNA structure is an siRNA specific for fibrosis or respiratory disease-related gene, particularly, amphiregulin or stratifin. In addition, the present invention relates to a pharmaceutical composition for preventing or treating fibrosis or respiratory diseases, containing an siRNA, a high-efficiency double-stranded oligo RNA structure containing the siRNA, or a nanoparticle containing the high-efficiency double-stranded oligo RNA structure, as an active ingredient. In addition, the present invention relates to a method of preventing or treating fibrosis or respiratory diseases, including administering the pharmaceutical composition for preventing or treating fibrosis or respiratory diseases to a subject in need thereof.

The present invention relates to extended release liquid compositions of guaifenesin. The extended release liquid compositions are in the form of suspensions which are ready-to-use or suspensions which are reconstituted from powder. It also relates to processes for the preparation of said extended release liquid compositions.

Provided are mucolytic agents represented by formula (Ia)-(Id):

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where the structural variables R.sub.1, R.sub.2, R.sub.5 and R.sub.6 are as defined herein. Also provided are a variety of methods of treatment which take advantage of the mucolytic properties of the compounds represented by formula (Ia)-(Id).

A method for producing P.sup.1,P.sup.4-di(uridine 5-)tetraphosphate (UP.sub.4U) that can avoid reduction of the synthetic efficiency without using UTP free is developed. A method for producing UP.sub.4U comprising reacting a phosphoric acid-activating compound represented by formula [II] or [III] with a phosphoric acid compound selected from the group consisting of UMP, UDP, UTP and a pyrophosphoric acid or a salt thereof (excluding UTP free) in water or a hydrophilic organic solvent, in the presence of a metal ion selected from the group consisting of an iron (II) ion, an iron (III) ion, a trivalent aluminum ion, a trivalent lanthanum ion, and a trivalent cerium ion. where, in the formula [II], R.sup.1 represents a uridyl group binding to the 5-position, X represents a heterocyclic group, and n represents an integer of 1 or 2, where, in the formula [III], X represents a heterocyclic group selected from the group consisting of an imidazolyl group, a benzimidazolyl group, and a 1,2,4-triazolyl group. ##STR00001##

In one embodiment, the invention provides a method of treating a subject suffering from a Mycobacterium infection by administering to the subject a therapeutically-effective amount of a degradative autophagy agonist or a secretory autophagy antagonist. In another embodiment, the invention provides a method of treating a subject suffering from one or more diseases selected from the group consisting of a Mycobacterium infection, an inflammatory disorder, an immune disorder, a cancer and a neurodegenerative disorder by administering to the subject a therapeutically-effective amount of a TBK-1 antagonist (e.g. BX795 or amlexanox). Related pharmaceutical compositions, diagnostic and screening assays and kits are also provided.