Wustman
Brandon Wustman, Princeton, NJ US
Patent application number | Description | Published |
---|---|---|
20100266571 | DOSING REGIMENS FOR THE TREATMENT OF LYSOSOMAL STORAGE DISEASES USING PHARMACOLOGICAL CHAPERONES - The present invention provides dosing regimens for administering pharmacological chaperones to a subject in need thereof. The dosing regimens can be used to treat disorders caused by improper protein misfolding, such as lysosomal storage disorders. | 10-21-2010 |
20110105560 | TREATMENT OF CNS DISORDERS ASSOCIATED WITH MUTATIONS IN GENES ENCODING LYSOSOMAL ENZYMES - Described is a method for treating an individual having a neurological disorder with an associated mutation or mutations in a gene encoding a lysosomal enzyme. Specifically, the individual is administered a specific pharmacological chaperone for the lysosomal enzyme which increases trafficking of the protein from the ER to the lysosome in neural cells, with or without concomitantly increasing enzyme activity in neural cells. Restoration of trafficking relieves cell stress and other toxicities associated with accumulation of mutant proteins. Restoration of enzyme activity relieves substrate accumulation and pathologies associated with lipid accumulation. In a specific embodiment, the neurological disorder is Parkinson's disease or parkinsonism which is associated with mutations in glucocerebrosidase. | 05-05-2011 |
Brandon Alan Wustman, San Diego, CA US
Patent application number | Description | Published |
---|---|---|
20110052613 | METHOD FOR THE TREATMENT OF NEUROLOGICAL DISORDERS BY ENHANCING THE ACTIVITY OF BETA-GLUCOCEREBROSIDASE - Provided is a method of increasing the stability of wild-type β-glucocerebrosidase. Also provided are methods of treating and/or preventing an individual having a neurological disease in which increased expression or activity of β-glucocerebrosidase in the central nervous system would be beneficial. This method comprises administering an effective amount of a pharmacologic chaperone for β-glucocerebrosidase, with the proviso that the individual does not have a mutation in the gene encoding β-glucocerebrosidase. Further provided are β-glucocerebrosidase inhibitors which have been identified as specific pharmacologic chaperones and which have been shown to increase activity of β-glucocerebrosidase in vivo in the central nervous system. | 03-03-2011 |
20150025109 | Method for the Treatment of Neurological Disorders by Enhancing the Activity of Beta-Glucocerebrosidase - Provided is a method of increasing the stability of wild-type β-glucocerebrosidase. Also provided are methods of treating and/or preventing an individual having a neurological disease in which increased expression or activity of β-glucocerebrosidase in the central nervous system would be beneficial. This method comprises administering an effective amount of a pharmacologic chaperone for β-glucocerebrosidase, with the proviso that the individual does not have a mutation in the gene encoding β-glucocerebrosidase. Further provided are β-glucocerebrosidase inhibitors which have been identified as specific pharmacologic chaperones and which have been shown to increase activity of β-glucocerebrosidase in vivo in the central nervous system. | 01-22-2015 |
Roger D. Wustman, Mason, OH US
Patent application number | Description | Published |
---|---|---|
20080305264 | THERMAL BARRIER COATING AND PROCESS THEREFOR - A thermal barrier coating and deposition process for a component intended for use in a hostile thermal environment, such as the turbine, combustor and augmentor components of a gas turbine engine. The TBC has a first coating portion on at least a first surface portion of the component. The first coating portion is formed of a ceramic material to have at least an inner region, at least an outer region overlying the inner region, and a columnar microstructure whereby the inner and outer regions comprise columns of the ceramic material. The columns of the inner region are more closely spaced than the columns of the outer region so that the inner region of the first coating portion is denser than the outer region of the first coating portion, wherein the higher density of the inner region promotes the impact resistance of the first coating portion. | 12-11-2008 |
Roger Dale Wustman, Mason, OH US
Patent application number | Description | Published |
---|---|---|
20090162209 | TURBINE ENGINE COMPONENTS WITH ENVIRONMENTAL PROTECTION FOR INTERIOR PASSAGES - A gas turbine blade comprises a base metal, a platform, an airfoil extending upwardly from the platform, a shank extending downwardly from the platform. The shank has an exterior wall and an internal passage, and the airfoil has a cooling flow channel inside the airfoil for flowing a cooling flow therethrough. The blade has a first chromide coating contacting the base metal of at least a portion of an interior surface of the shank and interdiffused therewith, wherein the first chromide coating does not have an aluminide coating deposited over it. The blade has a second chromide coating contacting the base metal of at least a portion of an interior surface of the airfoil and interdiffused therewith. A method for preparing a gas turbine blade comprises the steps of applying chromide coatings, sealing the interior passages of the shank and airfoil and applying an aluminide or platinum aluminide coating and an optional ceramic layer on the airfoil. | 06-25-2009 |
20090162562 | Methods for Applying Thermal Barrier Coating Systems - Methods for coating a substrate includes depositing on the substrate, a inner bond coat layer of a bond coat composition comprising, in weight percent, 14-20% Cr, 5-8% Al, 8-12% Co, 3-7% Ta, 0.1-0.6% Hf, 0.1-0.5% Y, up to about 1% Si, 0.005-0.020% Zr, 0.04-0.08% C, 0.01-0.02% B, with a remainder including nickel (Ni) and incidental impurities, wherein the bond coat composition is substantially free of rhenium; forming an aluminum-containing layer overlying the inner bond coat layer; and, optionally, depositing a thermal barrier coating composition overlying the aluminum-containing layer. | 06-25-2009 |
20090162692 | Coated Superalloy Articles - Coated superalloy article includes a bond coat comprising an inner bond coat layer disposed on the first surface being formed by deposition of a bond coat composition comprising, in weight percent, 14-20% Cr, 5-8% Al, 8-12% Co, 3-7% Ta, 0.1-0.6% Hf, 0.1-0.5% Y, up to about 1% Si, 0.005-0.020% Zr, 0.04-0.08% C, 0.01-0.02% B, with a remainder including nickel (Ni) and incidental impurities, wherein the bond coating composition is substantially free of rhenium. An aluminum-containing layer overlies the inner bond coat layer. Optionally, a thermal barrier coating overlies the aluminum-containing layer, wherein the thermal barrier coating, if present, is formed by deposition of a thermal barrier coating composition. | 06-25-2009 |