Abstract: The present invention relates to an isolated nucleic acid molecules encoding mutant lama2 gene of zebrafish, mutant zebrafish having mutations in the lama2 gene, fish models containing mutant zebrafish, and uses of the fish models.

Abstract: A fuel cell assembly (100) comprising: an enclosure (120) for mounting a fuel cell stack (110) therein, the enclosure comprising an air flow path (160) extending between an air inlet (180) and an air outlet (190); and a fuel cell stack (110) having a plurality of cathode air coolant paths extending between a first face (111) and an opposing second face (112) of the stack, wherein the fuel cell stack is mounted within the enclosure to provide a first tapering air volume (140) between the first face of the stack and a first side wall (121) of the enclosure and a second tapering air volume (150) between the second face of the stack and a second opposing side wall (122) of the enclosure.

Abstract: The pedal drive mechanism comprises a pedal arm immovably affixed across the secant of a drive gear, with the drive gear and attached pedal arm rotating through a partial revolution during operation. The drive gear meshes with a smaller pinion gear installed concentrically on the drive wheel axle. The pedal arm extends generally upwardly in its initial position prior to actuation of the mechanism, with operation requiring a rearward and downward kick or push. This is a natural motion for the operator of a scooter type vehicle, and the downward force also transfers a greater force to the drive wheel(s) for better traction during starting and operation. A return spring rotates the pedal arm, drive gear, and pinion gear back to their original starting positions when pressure is released on the pedal, with a one-way clutch disengaging the pinion gear from the drive wheel(s) during the pedal return stroke.

Abstract: Magnetic separator devices that are useful in separating finely divided solids in the presence of liquids, vapors, and gases that are hazardous, that is, they may be corrosive, flammable, toxic, or a combination of such hazards, and the use of such devices in processes for the manufacture of chlorosilanes.

Abstract: There are disclosed compound of Formula (I) or (II) wherein A1, A2, A3, A4 and A5, which may be the same or different, represent N or CR1, R9 represents -L-R3, in which L is a single bond or a linker group and R3 represents hydrogen or a substituent and in addition, when an adjacent pair of A1-A4 each represent CR1, then the adjacent carbon atoms, together with their substituents may form a ring B, when A5 represents CR1, then A5 and N—R9, together with their substituents may form a ring C, or a pharmaceutically acceptable salt thereof, in the manufacture of a medicament for the therapeutic and/or prophylactic treatment of Duchenne muscular dystrophy, Becker muscular dystrophy or cachexia.

Abstract: The present invention provides an inspection system for inspecting a surface of an optical specimen. The inspection system includes an optical testing device having a main body and an optical axis. The optical testing device includes an optical imaging system housed in the main body. The optical imaging system includes imaging components for acquiring a microscope visual image and for acquiring at least one interference fringe image of the surface of the optical specimen. The optical testing device also includes a translational mechanism housed in the main body and configured to allow linear movement of the optical imaging system and to prevent off-axis movement of the optical imaging system.

Abstract: Compounds of general formula (I) wherein R is phenyl optionally substituted with one or more halo substituents; and their pharmaceutically acceptable salts, hydrates, solvates, complexes or prodrugs are useful in orally administrable compositions for the treatment of allergic diseases such as asthma, allergic rhinitis and atopic dermatitis.

Abstract: In an automatically switched optical network operating according to a wavelength plan, the wavelengths are assigned to an optical path based on availability, performance and SRS wavelength coupling reduction. First, the wavelengths are grouped in static bins based on their reach versus cost performance, and each bin assumes a ?Q of a middle wavelength. Then, the bins are moved into subsets of dynamic bins, constructed using bin constraints that account for the particulars of the respective optical path. The path is characterized taking into account the wavelength currently accessing at the end nodes, and the wavelength tandeming through the end nodes. Wavelength selection starts with the bins that satisfy the maximum number of constraints, and the wavelengths are checked sequentially against wavelength constraints; relaxed constraints are also applied when it is not possible to exactly satisfy one or more constraints.

Abstract: A three seal sachet and method of manufacturing the sachet. The sachet includes a film having first and second longitudinal sheet edges and opposed first and second sheet faces. The film is folded to form opposed first and second sachet faces where the film is sealed to itself along marginal portions of the first and second longitudinal sheet edges on the first sheet face thereby forming a tube with a longitudinal seal on the second sachet face. First and second transverse seals bond the film along the marginal portions of the first and second transverse edges of the sheet, thereby closing the tube at each end. The sachet also includes a dispensing device disposed on the first or second face. The disposed device includes a flange which is inserted into the first sachet face and secures the device to the sachet and a dispensing orifice enabling product to be dispensed from the sachet.

Abstract: A pressurized metered dose dispenser (100) for dispensing an aerosol formulation comprising particles of a medicament suspended in liquefied propellant, optionally in combination with one or more excipients, the dispenser comprising an aerosol container (1) equipped with a metered dose valve (10), where a formulation chamber is defined in part by the internal walls of the container, and wherein the dispenser further comprises a porous, fluid permeable, particulate semi-permeable body (40) located within the formulation chamber adjacent to the metered dose valve.

Abstract: The present invention provides an apparatus (100) for forming circumferential grooves (14) in the exterior surface of a linear member (10), such as a tube. The grooves are formed by rotating a cylindrical cutting element (120) around the tube such that cutting means in the cylindrical cutting element form the grooves. One rotation of the cylindrical cutting element causes multiple grooves to be formed in the surface of the tubes.

Abstract: A fluid flow field plate for use in a fuel cell, the plate comprising a first plurality of channels formed in a first surface thereof and extending across the first surface in a predetermined pattern, the plate having a folded region along a lateral edge, the folded region comprising a plenum and an interface region, the plenum having a longitudinal axis substantially parallel to an edge of the plate, the interface region comprising two adjacent and facing portions of the first surface.

Abstract: A service station facility for replenishing various motivational energy sources onboard different types of automotive vehicles is disclosed herein. In one embodiment, the service station facility includes a rack, replaceable fuel tanks, a service module, and an electronic computer control system. The replaceable fuel tanks are stocked on the rack and substantially filled with various fluids, such as hydrogen, which are utile as motivational energy sources within fuel-operated automotive vehicles. The service module is mounted on the rack, and the electronic computer control system is connected in electrical communication with the service module. In this configuration, the service module is controllably operable to receive a depleted replaceable fuel tank from a fuel-operated automotive vehicle and also selectively deliver one of the filled replaceable fuel tanks onboard the automotive vehicle.

Abstract: A spring-applied parking brake with manual release and reapply. A gear box fixed relative to the rear wall of a cylinder has a manual crank. A pushrod extending through the cylinder and gear box is connected to provide braking force. A threaded collet surrounding helical threads on the pushrod has flexible radially expandable tines. When the gear box is manually cranked, the pushrod is rotated and due to interaction of the threads on the inside of the collet and on the pushrod, the pushrod is moved axially releasing or reapplying the brake.

Abstract: A gasket for sealing internal surfaces of a fuel cell and formed of compressible material, the gasket comprising a first sealing surface and a second sealing surface for providing a fluid seal against opposing faces of a first fluid flow field plate and a second fluid flow field plate respectively, the gasket further comprising a third sealing surface for sealing against an outer perimeter region of a first surface of a membrane electrode assembly, the third sealing surface being entirely enclosed within a boundary defined by an inner perimeter of the second sealing surface.

Abstract: The data transmitted from a first station is transmitted at a first data rate. The data transmitted from a second station is transmitted at a second data rate. The second data rate is lower than the first data rate. In view of both the difference in data rates and the synchronisation between radio stations, a separate synchronisation word is not required to be sent from the second station to the first station on the uplink portion; instead, and because the uplink slots of a given frame are synchronised between transceiving stations, the start of the uplink payload slot can comprise signalling information which simply acts to signify the start of transmission of uplink data from the second station.

Abstract: An RF receiver comprises a signal processor arranged to perform a method of decoding data contained within a signal that comprises a set of slots, at least one said slot comprising a preamble portion and a payload portion and being transmitted at a predetermined transmission frequency. The signal processor is arranged to perform a first process to derive timing data from the preamble portion and perform a second process to extract information from the payload portion, the second process being triggered from said timing data derived from the first process. The preamble portion comprises at least a first sequence of data and a second sequence of data, and the second sequence is the inverse of the first sequence. In preferred embodiments the first process comprises identifying a transition between said first and second sequences of data and deriving said timing data from the identified transition.

Abstract: Systems and methods for providing digital rights management services are disclosed. Such a system includes a service program that provides a processing framework for performing a digital rights management service, such as publishing or licensing rights managed digital content. A plurality of plug-in components are provided, each of which performs a respective task associated with the digital rights management service. The plug-in components are integrated into the processing framework according to predefined sets of interface rules.