Abstract: The present disclosure relates to compounds of formula (I) and pharmaceutical compositions and their use in reducing Widely Interspaced Zinc Finger Motifs (WIZ) expression levels, or inducing fetal hemoglobin (HbF) expression, and in the treatment of inherited blood disorders (e.g., hemoglobinopathies, e.g., beta-hemoglobinopathies), such as sickle cell disease and beta-thalassemia.

Abstract: The present invention relates to a process for the preparation, also on an industrial scale, of indocyanine green of formula (I) (ICG, 1 H-benz[e]indole, 2-[7-[1,3-dihydro-1,1-dimethyl-3-(4-sulfobutyl)-2H-benz[e]indol-2-ylidene]-1,3,5-heptatrienyl]-1,1-di-methyl-3-(4-sulfobutyl) hydroxide, inner salt, sodium salt, CAS RN 3599-32-4) with a total impurity content <0.5% and % and single impurity <0.10%, MeOH free, purity determined by a new analytical method HPLC at the wavelength of 254 nm, and the related composition with stable NaI, which is water soluble and has NaI content <2.5%.

Abstract: A connection node assembly for use in connecting an upper prefabricated building module to a lower module. Hollow vertical edge supports of the building modules have top and bottom connection parts. A connecting bolt can be passed through the top part of an upper vertical support in the upper building module and seated within the bottom part of that vertical support with the shank extending downward. The shank engages a threaded aperture in a connecting nut secured within the top part of a corresponding lower vertical support in the lower building module. Diaphragm plates sandwiched between the upper and lower vertical supports are used to tie horizontally placed modules together.

Abstract: A system for detecting tumoral masses in an unknown tissue is described, that can be coupled to an MRI system for receiving a number of unknown tissue scans, each scan comprising a corresponding plurality of MRI images relating to a group of voxels, each voxel being associated with a corresponding portion of unknown tissue, the MRI images of each scan being indicative of values of at least a corresponding initial parameter. The detection system determines for each voxel a corresponding probability value, indicative of the probability of the corresponding portion of unknown tissue including a corresponding tumoral portion. Furthermore, the detection system determines, for a plurality of voxel groups, a corresponding group aggressiveness value, indicative of the aggressiveness of the tumoral mass formed by the tumoral portions present in the corresponding portions of unknown tissue.

Abstract: An engine assembly defining an axial direction (A) and including a gearbox, an engine core including at least one rotor, and a flexible coupling shaft having a first end and a second end along the axial direction (A). The first end of the flexible coupling shaft is connected to the engine core and the second end of the flexible coupling shaft is connected to the gearbox. A damper system is positioned at the second end of the flexible coupling shaft. The damper system is configured to reduce vibrations to the flexible coupling shaft during operation of the engine assembly.

Abstract: A device for sludge treatment comprises a supporting frame of an external casing defining a treatment chamber provided with one inlet opening of the sludge to be treated; one discharge opening of the treated sludge; at least one auger element housed within the treatment chamber, and adapted to move the sludge to be treated along a direction of advancement; and filtration means located internally to the treatment chamber and surrounding the auger element; at least one anodic element associated with the auger element, and application means for applying a potential difference between the anodic element and the filtration means, generating an electric field adapted to interact with the sludge to be treated to produce an electro-osmotic process.

Abstract: The invention relates to a method for laser-based splicing of a glass fiber (1) onto an optical component (3), comprising the following steps: arranging both surfaces to be spliced substantially parallel to each other and at a predefined distance from each other; and aiming a laser beam (4) at the optical component (3). In order to specify an improved method in which the properties of the joining partners are maintained to the greatest extent during splicing, which exhibits high reproducibility and in particular is suitable for splicing joining partners of different cross-sections, the invention proposes that the angle of incidence of the laser beam (4) on the surface of the optical component be between 15° and 45°.

Abstract: A gas treating apparatus may include a reaction chamber configured to process a gas supplied from an outside by a plasma, the processed gas containing a nitrogen oxide, and a nitrogen oxide reduction apparatus connected to the reaction chamber. The nitrogen oxide reduction apparatus includes a cooling unit configured to cool the processed gas to a temperature lower than a nitrogen oxide generation temperature.

Abstract: Audio encoder for encoding audio input data to obtain audio output data includes an input interface for receiving a plurality of audio channels, a plurality of audio objects and metadata related to one or more of the plurality of audio objects; a mixer for mixing the plurality of objects and the plurality of channels to obtain a plurality of pre-mixed channels, each pre-mixed channel including audio data of a channel and audio data of at least one object; a core encoder for core encoding core encoder input data; and a metadata compressor for compressing the metadata related to the one or more of the plurality of audio objects, wherein the audio encoder is configured to operate in at least one mode of the group of two modes.

Abstract: The present invention concerns the use of the microorganisms species Pseudomonas trivialis, Pseudomonas lurida, Phaeophlebiopsis sp., Periconia macrospinosa for preventing and/or treating Fusarium head blight in cereal plants and/or grains.

Abstract: It is provided a method for gene repair in primary human muscle stem cells (satellite cells) in vitro comprising the following steps: providing a sample of an isolated muscle-fiber containing tissue sample collected from at least one patient with a monogenic muscle disease, wherein the monogenic muscle disease is caused by at least one mutation in at least one gene encoding for at least one muscle protein; isolating and cultivating primary stem cells from said muscle-fiber containing tissue sample, and correcting the at least one mutation in the at least one gene encoding for at least one muscle protein in the cultivated primary stem cells by targeted modification of the at least one mutation by gene editing using CRISPR/Cas-based tools.

Abstract: A hydrogen cooled pressure packer, systems, and methods of operation are provided. The pressure packer can include a flange portion and a plurality of packing cups coupled to the flange portion. One or more packing cups of the plurality of packing cups include at least one injection channel extending therethrough and terminating in at least one injection port. The pressure packer can also include a seal abutted with at least one packing cup of the plurality of packing cups. The pressure packer can be configured for use in a hydrogen compressor operable within a hydrogen vehicle refueling facility.

Abstract: A safety switch comprises a switching device (2) and a driving device (3), wherein the switching device houses locking/releasing means (23) of the driving device (3) to prevent unauthorized opening of the access. The driving device (3) comprises an interaction element (17) adapted to interact with the locking/releasing means (23), these latter being provided with a locking pin (24) movable between an inoperative unlocking position and an operative locking position, the interaction element (17) being provided with a seat (25) for the insertion of one end of the locking pin (24). The locking/releasing means (23) comprises a locking element (40) adapted to translate to engage the interaction element (17) in a locking position wherein the interaction element (17) is locked on the switching device (2) and in a holding position wherein the interaction element (17) is removable.

Abstract: A gas (or steam) turbine is disclosed including: a rotor with at least one array of rotor blades, a stator with a casing and a shroud ring; the shroud ring extends around the array of blades rotor and the casing extends around the shroud ring. The shroud ring has radial size independent from temperature due to its material and is movably coupled with the casing so to allow the casing of the stator to thermally expand and contract during operation of the turbine while maintaining the shroud ring radial size. Also, the rotor thermally expands and contracts during operation of the turbine, and, at working temperature, tip regions of the rotor blades are in close proximity to an inner region of the shroud ring so that clearance is small or zero at working condition.

Abstract: Methods and devices for two nodes to authenticate each other as credentialed by a group of autonomous specialized nodes, without involving the group or involving a centralized certificate manager or authenticator. The method may involve a first node and a second node using bilinear pairing operations involving their respective identifiers and secret points to derive the same session key. Provided the secret points and identifiers were obtained from the group using the group private key, the bilinear pairing operation leads to generation of the same session key at each of the two nodes, thereby authenticating their respective credentials and enabling trusted communications between the two nodes.

Abstract: A circuit for use, e.g., as current sense amplifier in a DC-DC converter in a hybrid vehicle includes a first input node and a second input node, configured to have an input voltage signal applied therebetween, a floating-ground input stage configured to operate between a first supply voltage and a second non-zero supply voltage and to convert into a current signal the input voltage signal applied between the first input node and the second input node. The circuit includes an output stage configured to receive the current signal from the floating-ground input stage and to convert the current signal back to an output voltage signal referred to ground. The output voltage referred to ground is a replica of the input voltage signal applied between the first input node and the second input node.

Abstract: Device for detecting biomolecules, comprising a container intended to be filled with a mixture comprising a biomolecule to be detected, a reagent bondable to the biomolecule and susceptible of emitting a first light radiation in fluorescence or chemiluminescence, and a plurality of magnetic beads chemically bondable to the biomolecule. The device comprises a first detector for detecting the first light radiation for detecting the biomolecule concentration, a first emitter for emitting a second light radiation for irradiating the magnetic beads, and a second detector for detecting a third light radiation consequently transmitted, reflected or emitted by the magnetic beads for detecting the quantity of magnetic beads. The device also comprises a logic control unit configured for calculating a correct concentration value of biomolecule on the basis of the above measurements of the light radiations.

Abstract: The present invention discloses a conjugate comprising an antigen (for example a saccharide antigen) covalently linked to a Pseudomonas aeruginosa PcrV carrier protein comprising an amino acid sequence which is at least 80% identical to the sequence of SEQ ID NO:1-4, wherein the antigen is linked (either directly or through a linker) to an amino acid residue of the P. aeruginosa PcrV carrier protein. The invention also discloses Pseudomonas aeruginosa PcrV proteins that contain glycosylation site consensus sequences.

Abstract: Described are a system, method, and computer program product for secure client device and consumer authentication. The method includes receiving a primary account number (PAN) of an account of a consumer from a merchant system and determining whether the PAN has been registered with a public key of a public-private key pair of a client device of the consumer. The method also includes, in response to determining that the PAN has been registered, transmitting a login request to a transaction processing system including a device identifier of the client device based on the PAN. The method further includes receiving a login response from the transaction processing system associated with successful authentication of the client device and, in response to receiving the login response, processing a transaction by generating a transaction authorization request message including the PAN to the transaction processing system.