Abstract: The present invention provides a method of treating NPP1 deficiency or NPP1-associated disease such as idiopathic infantile arterial calcification (IIAC), pseudoxanthoma elasticum, vascular calcification in chronic kidney disease (VCCKD), insulin resistance, hypophosphatemic rickets, myocardial ischemia, joint calcification, angioid streaks, and ossification of the posterior longitudinal ligament of the spine. The present invention provides a method for treating tissue calcification by administering soluble NPP1 to produce a transient increase in serum pyrophosphate levels.

Abstract: The present invention provides methods of treating LAL deficiency comprising administering to a mammal a therapeutically effective amount of lysosomal acid lipase with an effective dosage frequency. Methods of improving growth and river function, increasing LAL tissue concentration, and increasing LAL activity in a human patient suffering from LAL deficiency are also provided.

Abstract: Methods for treating the central nervous system (CNS) in a human patient with mucopolysaccharidosis (MPS) MB who has an intact blood brain barrier, comprising intravenous administration of recombinant NaGlu.

Abstract: The present invention provides compositions of recombinant human lysosomal acid lipase having particular glycosylation patterns for internalization into target cells, a vector containing the nucleic acid encoding human lysosomal acid lipase, a host cell transformed with the vector, pharmaceutical compositions comprising the recombinant human lysosomal acid lipase and method of treating conditions associated with lysosomal acid lipase deficiency.

Abstract: Single molecules useful in vaccine compositions, and methods of making and using the same, are described.

Abstract: The present invention provides methods of treating LAL deficiency comprising administering to a mammal a therapeutically effective amount of lysosomal acid lipase with an effective dosage frequency. Methods of improving growth and liver function, increasing LAL tissue concentration, and increasing LAL activity in a human patient suffering from LAL deficiency are also provided.

Abstract: The present invention provides a method of treating NPP1 deficiency or NPP1-associated disease such as idiopathic infantile arterial calcification (IIAC), pseudoxanthoma elasticum, vascular calcification in chronic kidney disease (VCCKD), insulin resistance, hypophosphatemic rickets, myocardial ischemia, joint calcification, angioid streaks, and ossification of the posterior longitudinal ligament of the spine. The present invention provides a method for treating tissue calcification by administering soluble NPP1 to produce a transient increase in serum pyrophosphate levels.

Abstract: A poultice application pad includes a generally planar poultice layer for application to a treatment area of a limb of an animal, and a backing sheet, adjacent to the poultice layer, which backing sheet supports and holds the form of the poultice layer when contacting the first side of the poultice layer to the treatment area to reduce application time and displacement of the mud/clay mixture beyond the treatment area. Also disclosed is a set of such poultice application pads stacked one on top of another within a sealable container.

Abstract: A system for automatically producing a brine solution having a concentration of a primary solute that is stable at a defined temperature comprises a controller, a mixing reservoir, a primary solute concentration sensor, and a storage tank. Methods for automatically producing solutions are described.

Abstract: The present invention is directed to isolated recombinant human ADA2 proteins, ADA2 biologically active fragments, and ADA2 fusion proteins. The proteins of the invention can be surprisingly used to restore ADA2 activity in subjects having loss-of-function mutations in ADA2 and increase differentiation of monocytes into macrophages, e.g., M2 macrophages, e.g., M2c macrophages, stimulate CD4+ T cell proliferation, and increase endothelial cell development. More specifically, the ADA2 proteins, ADA2 biologically active fragments, and ADA2 fusion proteins of the invention can be used to treat subjects having ADA2-associated diseases or disorders, including but not limited to, polyarteritis nodosa, Sneddon Syndrome, vasculitis, ischemic stroke, hemorrhagic stroke, lacunar stroke, aneurysm in the celiac artery, skin rash, skin necrosis, livedo racemosa, hepatosplenomegaly, organ failure, retinal artery occlusion, optic nerve atrophy, diplopia, cranial nerve palsy, strabismus, and low serum IgM.

Abstract: The present disclosure includes methods of making a brine solution that include recirculating brine solution to a salt and water hopper from a holding tank. The present disclosure also includes an apparatus for making a solution that includes at least one nozzle assembly positioned inside the lower half of a hopper. The at least one nozzle assembly includes a manifold having at least two nozzles spaced apart. Each nozzle has one or more nozzle outlets that are directed away from the bottom of the hopper (e.g., toward the top of the hopper).

Abstract: The present invention provides compositions of recombinant human lysosomal acid lipase having particular glycosylation patterns for internalization into target cells, a vector containing the nucleic acid encoding human lysosomal acid lipase, a host cell transformed with the vector, pharmaceutical compositions comprising the recombinant human lysosomal acid lipase and method of treating conditions associated with lysosomal acid lipase deficiency.

Abstract: Recombinant human lysosomal acid lipase (rhLAL) containing an N-terminal truncation, a composition of truncated recombinant human LAL (TLAL), an isolated mixture comprising TLAL and at least one other form of rhLAL are disclosed. A method of purifying TLAL from a mixture of LAL proteins, pharmaceutical compositions comprising TLAL and methods of producing TLAL are further disclosed.

Abstract: The present invention provides compositions comprising an isolated mixture of recombinant human NaGlu proteins in which a substantial amount of the NaGlu proteins in the mixture has increased levels of phosphorylated mannose that confer the proteins to be efficiently internalized into human cells. The present invention also provides methods of producing such mixture of NaGlu proteins, vectors used in transgenesis and expression, host cells harboring such vectors, and methods of isolating and purifying the mixture of NaGlu proteins. The invention further provides methods of treating NaGlu associated diseases.

Abstract: Methods for treating the central nervous system (CNS) in a human patient with mucopolysaccharidosis (MPS) MB who has an intact blood brain barrier, comprising intravenous administration of recombinant NaGlu.

Abstract: The present invention provides a novel fusion polypeptide containing a catalytic domain of NPP1 fused to a targeting moiety, nucleic acids encoding the fusion polypeptide, a vector containing the nucleic acid integrated thereinto, a host cell transformed with the vector and pharmaceutical compositions comprising the fusion polypeptide.

Abstract: The present invention provides compositions comprising an isolated mixture of recombinant human NaGlu proteins in which a substantial amount of the NaGlu proteins in the mixture has increased levels of phosphorylated mannose that confer the proteins to be efficiently internalized into human cells. The present invention also provides methods of producing such mixture of NaGlu proteins, vectors used in transgenesis and expression, host cells harboring such vectors, and methods of isolating and purifying the mixture of NaGlu proteins. The invention further provides methods of treating NaGlu associated diseases.

Abstract: The present invention provides a novel fusion polypeptide containing a catalytic portion of NPP1 fused to a targeting moiety, nucleic acids encoding the fusion polypeptide, a vector containing the nucleic acid integrated thereinto, a host cell transformed with the vector and pharmaceutical compositions comprising the fusion polypeptide.

Abstract: A sealant hose having retained therein a sealant or other fluid material for insertion into a closed system such as an air conditioner or refrigeration system. The hose includes at one end thereof an inlet valve and at the other end thereof an outlet valve. The inlet valve and the outlet valve may be connected to the hose by use of a collar or other securing member. The inlet valve comprises a needle valve for attachment a manifold gauge. The outlet valve is adapted to attach to an air conditioner or refrigeration service port.

Abstract: The present invention provides methods of treating LAL deficiency comprising administering to a mammal a therapeutically effective amount of lysosomal acid lipase with an effective dosage frequency. Methods of improving growth and liver function, increasing LAL tissue concentration, and increasing LAL activity in a human patient suffering from LAL deficiency are also provided.