Abstract: An aircraft gas turbine engine includes a heat exchanger module, and a core engine including an intermediate-pressure compressor, a high-pressure compressor, a high pressure turbine, and a low-pressure turbine. The high-pressure compressor is connected to the high-pressure turbine by a first shaft, and the intermediate-pressure compressor is connected to the low-pressure turbine by a second shaft. The heat exchanger module includes a central hub and heat transfer elements extending radially from the central hub and spaced in a circumferential array, for transferring heat energy from a fluid within the heat transfer elements to an inlet airflow passing over the heat transfer elements prior to entry of the airflow into an inlet to the core engine. The gas turbine engine further includes a first electric machine connected to the first shaft and positioned downstream of the heat exchanger module, and a second electric machines connected to the second shaft.

Abstract: An aircraft turbofan gas turbine engine includes a fan assembly, a compressor module, and a turbine module. An electric machine positioned downstream of the fan assembly is rotationally connected to the turbine module. The fan assembly is in fluid communication with the compressor module by an intermediate duct and includes a highest pressure fan stage having a plurality of fan blades defining a fan diameter. The compressor module includes a lowest pressure compressor stage having a row of rotor blades. An intermediate flow axis is defined joining a radially outer tip of a trailing edge of one of the fan blades of the highest pressure fan stage, and a radially outer tip of a leading edge of one of the rotor blades of a leading edge of a lowest-pressure compressor blade. An intermediate flow axis angle and the intermediate flow axis angle is from ?20 to ?30 degrees.

Abstract: A turbofan gas turbine engine comprises, in axial flow sequence, a heat exchanger module, a fan assembly, a compressor module, a turbine module, and an exhaust module. The fan assembly comprises a plurality of fan blades defining a fan diameter (D). The heat exchanger module is in fluid communication with the fan assembly by an inlet duct, and the heat exchanger module comprises a plurality of radially-extending hollow vanes arranged in a circumferential array with a channel extending axially between each pair of adjacent hollow vanes. The heat exchanger module has a square axial cross-sectional profile, where a side length of the square cross-section is D.

Abstract: The present invention provides methods and compositions of treating Friedreich's ataxia (FRDA) based on administering an mRNA encoding a frataxin protein.

Abstract: The present invention provides methods and compositions of treating Friedreich's ataxia (FRDA) based on administering an mRNA encoding a frataxin protein.

Abstract: The invention relates to a portable material inspection device, with a light source, a polarizer, and an analyzer. The light source emits light through the polarizer to a material and the light reaches the analyzer. The angle or position of the incident emitted light, either polarized or unpolarized is freely adjustable. The inspection device may have an waterproof and dust resistant external shell and be relatively small, less than 7 inches by 5 inches by 2 inches.

Abstract: The present invention provides methods and compositions of treating Friedreich's ataxia (FRDA) based on administering an mRNA encoding a frataxin protein.

Abstract: Methods and systems are provided for mitigating excessive exhaust pressures in an engine. In one example, a method may include adjusting an intake throttle responsive to exhaust pressure upstream of an exhaust turbine being higher than a threshold without reducing boost level. In this way, boost pressures may be maintained while reducing exhaust pressures.

Abstract: An actuation arrangement for effecting actuation of pivotable vane, for example, variable inlet guide vane in gas turbine engine. The actuation arrangement includes an actuator having a support plate and pivotable plate. The pivotable plate is fixedly connected to pivotable vane and pivotable plate and pivotable vane are pivotable about a pivot axis. The actuator further includes at least one actuation element having a length extending from support plate to proximal a transverse edge portion of pivotable plate substantially perpendicular to pivot axis. The or each actuation element is fixedly connected to each of the support and pivotable plates and formed of a material that changes dimension upon application of external energy. Thus, upon application of external energy, for example, heat, a change in length of the or each actuation element occurs such that change in length effects pivoting of pivotable plate and pivotable vane about the pivot axis.

Abstract: Methods and systems are provided for mitigating excessive exhaust pressures in an engine. In one example, a method may include adjusting an intake throttle responsive to exhaust pressure upstream of an exhaust turbine being higher than a threshold without reducing boost level. In this way, boost pressures may be maintained while reducing exhaust pressures.

Abstract: An actuation arrangement for effecting actuation of pivotable vane, for example, variable inlet guide vane in gas turbine engine. The actuation arrangement includes an actuator having a support plate and pivotable plate. The pivotable plate is fixedly connected to pivotable vane and pivotable plate and pivotable vane are pivotable about a pivot axis. The actuator further includes at least one actuation element having a length extending from support plate to proximal a transverse edge portion of pivotable plate substantially perpendicular to pivot axis. The or each actuation element is fixedly connected to each of the support and pivotable plates and formed of a material that changes dimension upon application of external energy. Thus, upon application of external energy, for example, heat, a change in length of the or each actuation element occurs such that change in length effects pivoting of pivotable plate and pivotable vane about the pivot axis.

Abstract: Disclosed herein are compositions and methods for treatment of spinal muscular atrophy (SMA). In certain embodiments, compounds are provided that increase full-length survival of motor neuron (SMN) protein production by an SMN2 gene.

Abstract: Disclosed herein are compositions and methods for treatment of spinal muscular atrophy (SMA). In certain embodiments, compounds are provided that increase full-length survival of motor neuron (SMN) protein production by an SMN2 gene.

Abstract: Disclosed herein are compositions and methods for treatment of spinal muscular atrophy (SMA). In certain embodiments, compounds are provided that increase full-length survival of motor neuron (SMN) protein production by an SMN2 gene.

Abstract: Aryl substituted thiazol-2-yl-piperidines and related compounds useful as modulators of survival motor neuron (SMN) protein production are provided herein. Without being bound to any particular theory it is believed the aryl substituted thiazol-2-yl-piperidines and related compounds provided herein act to increase production of the SMN2 form of survival motor neuron protein. These compounds are useful for treating spinal muscular atrophy. Pharmaceutical compositions containing a carrier and one or more of the aryl substituted thiazol-2-yl-piperidine or related compounds described herein are also provided. Methods of treating spinal muscular atrophy are also provided by this disclosure.

Abstract: A metal channel for positioning and retaining metal studding and wall panels to form partition walls, comprising a central web with retaining flange portions along its opposite sides. The web is segmented, being divided along its length into discrete segments by transverse slots. One of the flange portions is similarly divided into segments by transverse slots aligned with the slots. Adjacent segments of the flange portions are however connected together by expansible bridges and optionally by frangible bridges. The other of the flange portions is provided with lines of perforation aligned with the slots and defining flexure zones. The channel is formed straight but can be bent into smooth curves by expansion of the expansible bridges.