Abstract: Method for enhancing in a mammalian cell the activity of an enzyme associated with Gaucher Disease by administering a competitive inhibitor of glucocerebrosidase in an amount effective to enhance the activity of the enzyme. Preferred compounds for use in the method are imino sugars and related compounds. In particular, C8-12-alkyl derivatives of N-alkyl-deoxynojirimycin, isofagomine compounds, and calystegine compoiunds are effective to enhance glucocerebrosidase activity.

Abstract: The invention relates to methods of enhancing normal melanocortin-4 receptor (MC4R) activity, and to enhancing activity of an MC4R having a mutation which affects protein folding and/or processing of the MC4R. The invention provides a method of treating an individual having a condition in which increased activity of an MC4R at the cell surface would be beneficial, for example in obesity, by administering an effective amount of a pharmacological chaperone for the MC4R. The invention provides MC4R pharmacological chaperones which enhance the activity of MC4R. The invention further provides a method of screening to identify pharmacological chaperones which enhance folding of an MC4R in the endoplasmic reticulum (ER), in order to enhance the activity of the MC4R at the cell surface.

Abstract: System and method of a compact X-ray imaging detector for dental imaging applications are disclosed. In one of the preferred embodiments, a compact X-ray point source is placed in patient's mouth whereas a X-ray detector is placed outside. The preferred improvement offers several advantages over prior art practices. One of the advantages is the much-reduced X-ray exposure to only areas to be imaged. An additional advantage is the improvement on image resolution due to geometric magnification associated with the location of the detector. The third substantial improvement is the enhanced contrast due to reduction of off-axis scattering. In another preferred embodiment, an X-ray imaging detection system is disclosed. The X-ray imaging system consists of a compact X-ray source that can be placed into a patient's mouth, an optical detector with scintillator, system control and an interfaced computer.

Abstract: The sliding blade microtome is a tissue specimen cutting device including a specimen syringe and an adjustable cutting mechanism for diagonally slicing the specimen. The specimen syringe is a cylindrical shaft having a first end and a second end for holding and supporting a specimen. The first end has a diameter that is smaller than the diameter of the shaft, forming a compressed lip for applying pressure on the specimen prior to slicing by the blade. The pressure used to compress the gelatin and the specimen holds the gelatin and the specimen in a sturdy position to slice the specimen to precision without chatter marks. The specimen is pushed out of the first end of the specimen syringe by a motor mechanism or a manually adjustable micrometer drive.

Abstract: Method for enhancing in a mammalian cell the activity of an enzyme associated with Gaucher Disease by administering a competitive inhibitor of glucocerebrosidase in an amount effective to enhance the activity of the enzyme. Preferred compounds for use in the method are imino sugars and related compounds. In particular, C8–12-alkyl derivatives of N-alkyl-deoxynojirimycin, isofagomine compounds, and calystegine compounds are effective to enhance glucocerebrosidase activity.

Abstract: Viewable images may be displayed from a substantially dark substrate. Light emitting material may be integrated into a substantially dark substrate. The light emitting material may be configured to emit visible light in response to absorption of ultraviolet light. The light emitting material may include a plurality of light emitting particles and each of the plurality of light emitting particles has a diameter less than about 500 nanometers. For example, emitting visible light from a substantially dark substrate may allow for images to be displayed with relatively high contrast. These high contrast images may appear clearer to a viewer and may result in less eye strain by a viewer.

Abstract: This application provides methods of improving protein replacement therapy by combining protein replacement therapy with active site-specific chaperones (ASSC) to increase the stability and efficiency of the protein being administered. The application further provides stable compositions comprising the purified protein and an ASSC, and methods of treatment by administering the compositions.

Abstract: Provided is a method for enhancing the activity of a protein, which protein when mutated, folds in an incorrect conformation in the endoplasmic reticulum, by contacting the protein with an effective amount compound which specifically binds to the protein and induces the protein to fold into a proper conformation (a specific chaperone). Also provided are methods for enhancing the stability and preventing the degradation of a protein by contacting the protein with an effective amount of a specific chaperone for the protein which stabilizes the protein in a proper conformation.

Abstract: The sliding blade microtome is a tissue specimen cutting device including a specimen syringe and an adjustable cutting mechanism for diagonally slicing the specimen. The specimen syringe is a cylindrical shaft having a first end and a second end for holding and supporting a specimen. The first end has a diameter that is smaller than the diameter of the shaft, forming a compressed lip for applying pressure on the specimen prior to slicing by the blade. The pressure used to compress the gelatin and the specimen holds the gelatin and the specimen in a sturdy position to slice the specimen to precision without chatter marks. The specimen is pushed out of the first end of the specimen syringe by a motor mechanism or a manually adjustable micrometer drive.

Abstract: In accordance with embodiments, viewable images can be created in glass. Viewable images may be created in glass by using a projector which projects ultraviolet light to excite light emitting material. Clear images may be created in glass because the size the light emitting particles in the glass is less than 400 nanometers. In embodiments, the visible illumination of a transparent substrate to display an image is possible, while the transparent substrate remains transparent. Accordingly, for example, drivers of automobiles may view images (e.g. map images) on their windshield while they are driving. As another example, window shoppers may view enhanced advertisements in the windows of stores that they are approaching.

Abstract: In accordance with embodiments, viewable images can be created in glass. Viewable images may be created in glass by using a projector which projects ultraviolet light to excite light emitting material. Clear images may be created in glass because the size the light emitting particles in the glass is less than 400 nanometers. In embodiments, the visible illumination of a transparent substrate to display an image is possible, while the transparent substrate remains transparent. Accordingly, for example, drivers of automobiles may view images (e.g. map images) on their windshield while they are driving. As another example, window shoppers may view enhanced advertisements in the windows of stores that they are approaching.

Abstract: In accordance with embodiments, viewable images can be created in glass. Viewable images may be created in glass by using a projector which projects ultraviolet light to excite light emitting material. Clear images may be created in glass because the size the light emitting particles in the glass is less than 400 nanometers. In embodiments, the visible illumination of a transparent substrate to display an image is possible, while the transparent substrate remains transparent. Accordingly, for example, drivers of automobiles may view images (e.g. map images) on their windshield while they are driving. As another example, window shoppers may view enhanced advertisements in the windows of stores that they are approaching.

Abstract: A symmetric dual-voltage charge pump and its control circuit generate bipolar output voltages. The charge pump converts a unipolar power source to a set of dual-voltage outputs of opposite polarity that are completely independent of each other. The charge pump includes two voltage-boosting transfer capacitors and two output capacitors. Two-phase operation generates an increased-magnitude output voltage of a negative polarity and another two phases of operation generate an increased output voltage of a positive polarity. The charge pump selectively charges one or both of the bipolar outputs with individual 2-phase charge cycles or with a sequence of charge cycles. When controlled by comparators with unequal reference voltages, the charge pump can force the bipolar outputs to unequal positive and negative voltages. Charge pumping is faster since only 2 phases are needed for charging either the positive or negative output.

Abstract: Method for enhancing in a mammalian cell the activity of an enzyme associated with Gaucher Disease by administering a competitive inhibitor of glucocerebrosidase in an amount effective to enhance the activity of the enzyme. Preferred compounds for use in the method are imino sugars and related compounds. In particular, C8-12-alkyl derivatives of N-alkyl-deoxynojirimycin, isofagomine compounds, and calystegine compoiunds are effective to enhance glucocerebrosidase activity.

Abstract: The present invention provides glucoimidazole (GIZ) and polyhydroxycyclohexenyl amine (PHCA) derivatives, methods of making them, and methods of use where the GIZ and PHCA derivatives have a short, flexible linker emanating from the corresponding position of the ring oxygen in a pyranose; and a lipophilic moiety connected to the linker and pharmaceutically acceptable salts thereof. More particularly, the present invention further provides a method for treating individuals having Gaucher disease by administering the novel GIZ or PHCA derivatives as “active-site specific chaperones” for the mutant glucocerebrosidase associated with the disease.

Abstract: The present invention provides novel hydroxy piperidine (HP) derivatives having (i) a positive charge at the position corresponding to the anomeric position of a pyranose ring; (ii) a short, flexible linker emanating from the corresponding position of the ring oxygen in a pyranose; and (iii) a lipophilic moiety connected to the linker and pharmaceutically acceptable salts thereof. The linker can be absent if the lipophilic moiety corresponds to a hydrocarbon chain with a linear length of 6 or more carbons. The present invention further provides a method for treating individuals having Gaucher disease by administering the novel HP derivative as “active-site specific chaperones” for the mutant glucocerebrosidase associated with the disease.

Abstract: Method for enhancing in a mammalian cell the activity of an enzyme associated with Gaucher Disease by administering a competitive inhibitor of glucocerebrosidase in an amount effective to enhance the activity of the enzyme. Preferred compounds for use in the method are imino sugars and related compounds. In particular, C8-12-alkyl derivatives of N-alkyl-deoxynojirimycin, isofagomine compounds, and calystegine compounds are effective to enhance glucocerebrosidase activity.

Abstract: A detector which may include the following: A flat base plate. An (N×M) array of detector tiles attaching on to the base plate, each said detector tile comprising an array of photo-sensors fabricated on a substrate having necessary circuitry. A plurality of data finger tiles attaching on to the said base plate, each data finger tile comprising a plurality of data lines. A plurality of scan finger tiles attaching on to the said base plate, each scan finger tile comprising a plurality of scan lines. An electrical interconnection network interconnecting the adjacent said detector tiles on their front surfaces. An electrical interconnection network connecting N units of the said detector tiles to a plurality of the said data finger tiles. An electrical interconnection network connecting M units of the said detector tiles to a plurality of the said scan finger tiles.

Abstract: In accordance with embodiments, viewable imagines can be created in glass. Viewable images may be created in or on glass (or other at least partially transparent substrate), by using microstructures to scatter light from a projector, while the glass maintains transparent or translucent properties. In embodiments, the microstructures are integrated into glass in patterns.

Abstract: In accordance with embodiments, viewable images can be created in glass. Viewable images may be created in glass by using a projector which projects ultraviolet light to excite light emitting material. Clear images may be created in glass because the size the light emitting particles in the glass is less than 400 nanometers. In embodiments, the visible illumination of a transparent substrate to display an image is possible, while the transparent substrate remains transparent. Accordingly, for example, drivers of automobiles may view images (e.g. map images) on their windshield while they are driving. As another example, window shoppers may view enhanced advertisements in the windows of stores that they are approaching.