The present invention relates to an antibody that specifically binding to acid sphingomyelinase (ASM) and a use thereof and, more specifically to an anti-ASM monoclonal antibody which has very high binding sensitivity and specificity to ASM protein and an excellent inhibitory effect on ASM activity, and to a use thereof in treatment/diagnosis of neurodegenerative diseases.

The invention provides methods for the synthesis of oligosaccharides comprising an aminooxy group. The invention further provides oligosaccharides comprising an aminooxy group, methods for coupling oligosaccharides comprising an aminooxy group to glycoproteins, and oligosaccharide-protein conjugates. Also provided are methods of treating a lysosomal storage disorder in a mammal by administration of an oligosaccharide-protein conjugate.

A method of treating pancreatic adenocarcinoma in an individual is disclosed. The method involves administering to the individual a therapeutically effective amount of a therapeutic agent comprising recombinant acid sphingomyelinase. In one embodiment, the therapeutic agent further includes one or more compositions selected from the group consisting of modified enzymes, fusion proteins and constitutively active mutants. In another embodiment, the therapeutic agent further includes a pharmaceutically acceptable excipient.

A reagent composition for use as a first reagent composition in a method of quantifying a small dense LDL cholesterol (sdLDL-C) in a sample, the method including causing the first reagent composition to react to the sample, and after the causing the first reagent composition to react to the sample, causing a second reagent composition for quantifying the sdLDL-C to react, thereby quantifying cholesterol in a remaining lipoprotein, the reagent composition includes a nonionic surfactant and has cholesterol esterase activity, cholesterol oxidase activity, and sphingomyelinase activity, and a contact angle between the reagent composition and a polyethylene terephthalate (PET) base material is equal to or more than 63.0 and equal to or less than 67.0.

The present invention provides, inter alia, compositions and methods for using CB1 cannabinoid receptor agonists, or other compounds capable of increasing endocannabinoids or endocannabinoid signaling, for treating and preventing lysosomal storage disorders in which lipid storage occurs (including, e.g., disorders associated with sphingomyelin accumulation). In particular embodiments, the present invention provides compositions and methods for treating such lysosomal storage disorders with one or more fatty acid amide hydrolase inhibitor alone or in combination with one or more additional agent.

The present invention includes compositions and methods for treating disease and disorders associated with pathological calcification or pathological ossification.

The present invention relates to a method for treating degenerative neurological disorders in a subject in need thereof, comprising administering to a subject in need thereof a therapeutically effective amount of a composition comprising an acid sphingomyelinase (ASM) activity inhibitor as an active ingredient.

The present disclosure provides methods of screening, diagnosing, monitoring and/or treating acid sphingomyelinase (ASM) disorders such as Niemann-Pick disease. In particular, the methods encompass techniques for improved diagnosis and/or treatment of an ASM disorder, for example using enzyme replacement therapy.

The present invention provides a oxazole compound represented by Formula (1), or a salt thereof:

##STR00001##

wherein R.sup.1 is an aryl group which may have one or more substituents; R.sup.2 is an aryl group or a nitrogen atom-containing heterocyclic group each of which may have one or more substituents; and W is a divalent group represented by Y.sup.1-A.sup.1- or Y.sup.2C(O) wherein Y.sup.1 is a group such as C(O), A.sup.1 is a group such as a lower alkylene group, and Y.sup.2 is a group such as a piperazinediyl group. The oxazole compound has a specific inhibitory action against phosphodiesterase 4.