Abstract: A method for synthesizing mesoporous silica microparticles comprising the steps of: preparing a sol from an ammonium catalyzed hydrolysis and condensation reaction of a pre-sol solution comprising a silica precursor and a structure directing agent dissolved in a mixed solvent system comprising an alcohol and water to produce mesoporous particles of silica with an average diameter of up to about 50 ?m; hydrothermally treating the particles to increase the pore size; treating the particles to remove residual structure directing agent; and further increasing the pore size using controlled dissolution.

Abstract: The invention provides a compound for use in treating or preventing a disease or condition selected from cystic fibrosis, chronic obstructive pulmonary disease (COPD), asthma, mild pulmonary disease, bronchitis, bronchiectasis, idiopathic bronchiectasis, allergic bronchopulmonary aspergillosis, sinusitis, rhinosinusitis, CFTR-related metabolic syndrome (CRMS), pancreatitis, idiopathic chronic pancreatitis and Sjörgren's syndrome, or for use in preventing male infertility caused by congenital absence of the vas deferens, in a patient by modulating CFTR activity, which compound is 9,10-dimethoxy-2-(2,4,6-trimethylphenylimino)-3-(N-carbamoyl-2-aminoethyl)-3,4,6,7-tetrahydro-2H-pyrimido[6,1-a]isoquinolin-4-one or a pharmaceutically acceptable acid addition salt thereof.

Abstract: Methods for enhancing the activity of an ion transporter in a cell and/or treating a channelopathy in a subject based on the administration or use of an effective amount of a fenretinide compound (fenretinide, a fenretinide analog, or a pharmaceutically acceptable salt thereof) and/or zinc are described. The methods are useful for restoring or increasing the cell surface expression of an ion transporter such as the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) that is mutated or defective. The fenretinide compound and/or zinc may be used in combination with ion transporter modulators, e.g., CFTR modulators, to further enhance the cell surface expression and activity of the ion transporter.

Abstract: The invention provides a compound for use in treating or preventing a disease or condition selected from cystic fibrosis, chronic obstructive pulmonary disease (COPD), asthma, mild pulmonary disease, bronchitis, bronchiectasis, idiopathic bronchiectasis, allergic bronchopulmonary aspergillosis, sinusitis, rhinosinusitis, CFTR-related metabolic syndrome (CRMS), pancreatitis, idiopathic chronic pancreatitis and Sjörgren's syndrome, or for use in preventing male infertility caused by congenital absence of the vas deferens, in a patient by modulating CFTR activity, which compound is 9,10-dimethoxy-2-(2,4,6-trimethylphenylimino)-3-(N-carbamoyl-2-aminoethyl)-3,4,6,7-tetrahydro-2H-pyrimido[6,1-a]isoquinolin-4-one or a pharmaceutically acceptable acid addition salt thereof.

Abstract: Methods for enhancing the activity of an ion transporter in a cell and/or treating a channelopathy in a subject based on the administration or use of an effective amount of a fenretinide compound (fenretinide, a fenretinide analog, or a pharmaceutically acceptable salt thereof) and/or zinc are described. The methods are useful for restoring or increasing the cell surface expression of an ion transporter such as the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) that is mutated or defective. The fenretinide compound and/or zinc may be used in combination with ion transporter modulators, e.g., CFTR modulators, to further enhance the cell surface expression and activity of the ion transporter.

Abstract: The invention provides a compound for use in treating or preventing a disease or condition selected from cystic fibrosis, chronic obstructive pulmonary disease (COPD), asthma, mild pulmonary disease, bronchitis, bronchiectasis, idiopathic bronchiectasis, allergic bronchopulmonary aspergillosis, sinusitis, rhinosinusitis, CFTR-related metabolic syndrome (CRMS), pancreatitis, idiopathic chronic pancreatitis and Sjörgren's syndrome, or for use in preventing male infertility caused by congenital absence of the vas deferens, in a patient by modulating CFTR activity, which compound is 9,10-dimethoxy-2-(2,4,6-trimethylphenylimino)-3-(N-carbamoyl-2-aminoethyl)-3,4,6,7-tetrahydro-2H-pyrimido[6,1-a]isoquinolin-4-one or a pharmaceutically acceptable acid addition salt thereof.

Abstract: A method for synthesizing mesoporous silica microparticles comprising the steps of: preparing a sol from an ammonium catalyzed hydrolysis and condensation reaction of a pre-sol solution comprising a silica precursor and a structure directing agent dissolved in a mixed solvent system comprising an alcohol and water to produce mesoporous particles of silica with an average diameter of up to about 50 ?m; hydrothermally treating the particles to increase the pore size; treating the particles to remove residual structure directing agent; and further increasing the pore size using controlled dissolution.

Abstract: The invention provides a compound for use in treating or preventing a disease or condition selected from cystic fibrosis, chronic obstructive pulmonary disease (COPD), asthma, mild pulmonary disease, bronchitis, bronchiectasis, idiopathic bronchiectasis, allergic bronchopulmonary aspergillosis, sinusitis, rhinosinusitis, CFTR-related metabolic syndrome (CRMS), pancreatitis, idiopathic chronic pancreatitis and Sjörgren's syndrome, or for use in preventing male infertility caused by congenital absence of the vas deferens, in a patient by modulating CFTR activity, which compound is 9,10-dimethoxy-2-(2,4,6-trimethylphenylimino)-3-(N-carbamoyl-2-aminoethyl)-3,4,6,7-tetrahydro-2H-pyrimido[6,1-a]isoquinolin-4-one or a pharmaceutically acceptable acid addition salt thereof.

Abstract: The present invention relates to pharmaceutical formulations for treating a respiratory tract infection or a pulmonary disease in an individual, comprising a calcium salt and a sodium salt, wherein the ratio of Ca+2 to Na+ is from about 4:1 (mole:mole) to about 16:1 (mole:mole). The invention also relates to methods of treating (including prophylactically treating) and reducing the spread of a respiratory tract infection, methods of treating (including prophylactically treating) a pulmonary disease or an acute exacerbation of a pulmonary disease, and methods of reducing the spread of an acute exacerbation of a pulmonary disease, comprising administering a pharmaceutical formulation that comprises a calcium salt and a sodium salt.

Abstract: The present invention relates to pharmaceutical formulations for treating a respiratory tract infection or a pulmonary disease in an individual, comprising a calcium salt and a sodium salt, wherein the ratio of Ca+2 to Na+ is from about 4:1 (mole:mole) to about 16:1 (mole:mole). The invention also relates to methods of treating (including prophylactically treating) and reducing the spread of a respiratory tract infection, methods of treating (including prophylactically treating) a pulmonary disease or an acute exacerbation of a pulmonary disease, and methods of reducing the spread of an acute exacerbation of a pulmonary disease, comprising administering a pharmaceutical formulation that comprises a calcium salt and a sodium salt.

Abstract: The invention relates to methods for treating bacterial infection of the respiratory tract, including pneumonias, such as ventilator-associated pneumonia, and to methods for treating ventilator-associated tracheobronchitis, comprising administering an effective amount of a salt formulation as an aerosol to the respiratory tract of an individual in need thereof. The formulations can also be used to reduce transmission of pathogen which can infect the respiratory tract, cause pneumonia or cause ventilator-associated tracheobronchitis.

Abstract: The present invention relates to a novel assay or screen for identifying compounds with potential therapeutic value for the treatment of protein trafficking diseases such as Cystic Fibrosis (CF) and nephrogenic diabetes insipidus (NDI). The usual approach involves expressing the mutant form of the gene in cells and assaying function in a multiwell format when cells are exposed to libraries of compounds. Although such functional assays are useful, they do not directly test the ability of a compound to correct defective trafficking of the protein. To address this a novel corrector screening assay for CF has been developed in which the appearance of the mutant protein at the cell surface is measured as the assay output. This assay was used to screen more than 3100 compounds. This novel screening approach to protein trafficking diseases is robust and general, and may enable the selection of molecules that can be translated rapidly to a clinical setting.

Abstract: A method for synthesising mesoporous silica microparticles comprising the steps of: —preparing a sol from an ammonium catalysed hydrolysis and condensation reaction of a pre-sol solution comprising a silica precursor and a structure directing agent dissolved in a mixed solvent system comprising an alcohol and water to produce mesoporous particles of silica with an average diameter of up to about 50 ?m; hydrothermally treating the particles to increase the pore size; treating the particles to remove residual structure directing agent; and further increasing the pore size using controlled dissolution.

Abstract: A process for preparing a mesoporous material comprises the step of preparing a sol and treating the sol material under supercritical fluid conditions. The treatment under supercritical fluid conditions forms an ordered mesoporous material. The sol may be applied to a substrate to form a mesoporous film and subsequently treating the film under supercritical fluid conditions. Alternatively the process may comprise directly treating the sol under supercritical fluid conditions to form a mesoporous powder material.

Abstract: The present invention relates to a method for labeling a membrane-localized protein by introducing a biotin target sequence tag into at least one loop domain of a membrane-localized protein. The method of the invention is useful for labeling an ion channel protein such as CFTR or mutants thereof. A method for identifying an agent which corrects protein misfolding of a membrane-localized protein is also provided.

Abstract: An image sensor which includes an integral color filter array and a method of making such sensor is disclosed. The sensor includes a semiconductor substrate having an overlying support layer with an optically planar surface, a plurality of spaced image pixels formed in the substrate; and an array of contiguous color filter elements overlying the planar surface whose top surfaces are coplanar and which have no overlap of color filter material between adjacent color filter elements.

Abstract: An image sensor which includes an integral color filter array and a method of making such sensor is disclosed. The sensor includes a semiconductor substrate having an overlying support layer with an optically planar surface, a plurality of spaced image pixels formed in the substrate; and an array of contiguous color filter elements overlying the planar surface whose top surfaces are coplanar and which have no overlap of color filter material between adjacent color filter elements.

Abstract: Disclosed herein are heat-activatable adhesive compositions comprising a water-soluble condensation polymer which is characterized by having a glycol component comprising one or more diols, preferably one of which is 1,4-bis(2-hydroxy-ethoxy)cyclohexane; and an acid component comprising greater than 15 and up to about 35 mole percent of at least one diacid or diester monomer having an iminodisulfonyl salt moiety, and from about 65 to about 85 mole percent of one or more other diacids or diesters. These adhesives are further characterized as having high bonding strengths at temperatures above 50.degree. C. They have been found useful in a variety of photographic materials, and particularly in image transfer film units.