Abstract: In some aspects, the disclosure relates to compositions and methods useful for inhibiting SOD1 expression in cells (e.g., cells of a subject). In some embodiments, the disclosure describes isolated nucleic acids engineered to express an inhibitory nucleic acid targeting endogenous SOD1 and an mRNA encoding a hardened SOD1 protein. In some embodiments, compositions and methods described by the disclosure are useful for treating Amyotrophic Lateral Sclerosis (ALS) in a subject.

Abstract: The invention provides an improved record making system, the system having a puck former having an outlet, the puck former being movable between an automatic position and a semi-automatic position and adapted to periodically produce a puck and to deliver the puck to the outlet. The system further has a record former having a puck receiver, the record former adapted, upon delivery of the puck to the puck receiver, to automatically produce a record. The record former is positioned relative to the puck former and adapted such that, when the puck former is in the automatic position, the outlet of the puck former is coterminous with the puck receiver of the record former. When the puck former is in the semi-automatic position, the puck receiver is accessible by an operator to permit manual puck feeding.

Abstract: The invention provides an improved record making system, the system having a puck former having an outlet, the puck former being movable between an automatic position and a semi-automatic position and adapted to periodically produce a puck and to deliver the puck to the outlet. The system further has a record former having a puck receiver, the record former adapted, upon delivery of the puck to the puck receiver, to automatically produce a record. The record former is positioned relative to the puck former and adapted such that, when the puck former is in the automatic position, the outlet of the puck former is coterminous with the puck receiver of the record former. When the puck former is in the semi-automatic position, the puck receiver is accessible by an operator to permit manual puck feeding.

Abstract: Aspects of the disclosure relate to methods of altering RNA splicing in a subject. In some embodiments, methods are provided for correcting splicing in a cell that contains a DYSF gene having a mutation that results in defective splicing.

Abstract: In some aspects, the disclosure relates to compositions and methods useful for inhibiting SOD1 expression in cells (e.g., cells of a subject). In some embodiments, the disclosure describes isolated nucleic acids engineered to express an inhibitory nucleic acid targeting endogenous SOD1 and an mRNA encoding a hardened SOD1 protein. In some embodiments, compositions and methods described by the disclosure are useful for treating Amyotrophic Lateral Sclerosis (ALS) in a subject.

Abstract: The improved record press is of the type wherein a relatively hot fluid is circulated through the platens to render the puck molten for molding and wherein a relatively cold fluid is circulated through the platens to cool the molten material for ejection. The improvement comprises the use of pressurized water as the relatively hot fluid.

Abstract: The present invention relates to methods to treat a neurodegenerative disease or disorder, e.g., a motor neuron disease in a subject, whereby the subject is administered a recombinant human Mullerian Inhibiting Substance (MIS) protein as disclosed herein, wherein the recombinant human MIS protein comprises a modified Kex cleavage site for increased cleavage. The recombinant human MIS protein can be produced from a pre-proprotein comprising a non-MIS leader sequence or a functional fragment thereof in place of the MIS leader sequence.

Abstract: In one aspect, the invention is directed to a method of identifying an individual that has amyotrophic lateral sclerosis (ALS) or is at risk of developing ALS comprising detecting one or more alterations in a profilin 1 (PFN1) sequence of an individual in need thereof compared to a wild type profilin sequence. In another aspect, the invention is directed to a method of identifying an individual that has amyotrophic lateral sclerosis (ALS) or is at risk of developing ALS comprising selectively sequencing a profilin 1 (PFN1) sequence of an individual in need thereof; and detecting one or more alterations in the PFN1 sequence of the individual. If the one or more alterations are detected then the individual has ALS.

Abstract: In one aspect, the invention is directed to a method of identifying an individual that has amyotrophic lateral sclerosis (ALS) or is at risk of developing ALS comprising detecting one or more alterations in a profilin 1 (PFN1) sequence of an individual in need thereof compared to a wild type profilin sequence. In another aspect, the invention is directed to a method of identifying an individual that has amyotrophic lateral sclerosis (ALS) or is at risk of developing ALS comprising selectively sequencing a profilin 1 (PFN1) sequence of an individual in need thereof; and detecting one or more alterations in the PFN1 sequence of the individual. If the one or more alterations are detected then the individual has ALS.

Abstract: An apparatus for conveying a substrate or sheet through a printing machine comprises a drive roller and a baffle assembly forming a path for the substrate past the drive roller. The baffle assembly includes an idler roller having an axle rotatably supported within the baffle assembly to cooperate with the drive roller to exert a nip force on the substrate. A nip spring connected to the baffle assembly bears against a bushing carrying the idler roller axle to exert a nip force on the idler roller. A nip force adjustment apparatus is mounted within the baffle assembly and is operable to apply an adjustment force on the bushing of the idler roller to augment the nip force generated by the nip spring.

Abstract: An improved method for monitoring stack quality in a finisher of a printer includes examining the net displacement of a compiling tray after each set has been ejected. A deviation from the normal increments of displacement for each set triggers a “tray full” condition which alerts a user to empty the tray.

Abstract: An improved sloped stack detection system for a multi-function finisher includes a reflective sensor which suspends the conveying of stapled sets to an output tray when staple build-up in small stapled sets causes the stacked output to become sloped.

Abstract: A heat exchanger useful for high temperature applications such as EGR cooling and fuel reformer applications comprises a tube bundle made up of a plurality of tubes, each having at least one end expanded to an enlarged polygonal cross-section, and having central portions with a generally smaller cross section. When the tubes are formed into a bundle, the enlarged end portions nest with one another and interstitial spaces are provided between the central portions of the tube. The enlarged end portions are preferably retained by a header ring having a multifaceted inner peripheral sidewall which is adapted to form brazed lap joints with the outward facing surfaces of the peripheral tubes end portions in the tube bundle. In one preferred arrangement, axially aligned enlarged portions are provided intermediate the ends of at least some of the tubes. These enlarged intermediate portions nest with one another and eliminate or reduce the need for baffle plates.

Abstract: A sheet folding apparatus, which may be used in conjunction with a printer or copier, includes a set of rollers forming a buckle zone, in which sheets are caused to buckle for creasing by a folding nip formed by the rollers. A blade is selectably positionable in and out of the buckle zone. When the blade is positioned in the buckle zone, any previously-folded portion of the sheet (such as in C-folding of the sheet) is prevented from being drawn away from the folding nip.

Abstract: Multiple sheet set compiling in a compiler of the seriatim output of printed sheets from a printer with a sheet entry position and a lateral sheet tamping system for laterally tamping the sheets being stacked in the compiler, in which the accumulated stack height of the sheets being compiled is estimated, and in response thereto the height of the top of the lateral sheet tamping system is raised to be maintained above the top of that rising (thicker) stack in the compiler, and lowered for the initial sheets stacking, and the compiler sheet entry position is also automatically raised in proportion to the increasing height of the stack of sheets being compiled in the compiler.

Abstract: A sheet folding apparatus, which may be used in conjunction with a printer or copier, includes a set of rollers forming a buckle zone, in which sheets are caused to buckle for creasing by a folding nip formed by the rollers. A blade is selectably positionable in and out of the buckle zone. When the blade is positioned in the buckle zone, any previously-folded portion of the sheet (such as in C-folding of the sheet) is prevented from being drawn away from the folding nip.

Abstract: It has been discovered that dysferlin is expressed in blood and that individuals who lack dysferlin in muscle also lack it in CD14(+) cells. Based on these discoveries, blood-based tests for dysferlin expression and uses of such tests are described.

Abstract: The invention features methods for treating a patient suffering from muscular dystrophy by administration of umbilical cord blood cells, e.g., by IV infusion.

Abstract: Mutations identified in dysferlin and methods to detect these mutations are described.

Abstract: A switch for use with an electrosurgical tool having a heatable tip connectable to a power source and a suction passage connectable to a suction source. The switch comprises a main body having a suction passage extending therethrough connectable to the suction passage of the tool. A cavity in the main body intersects the suction passage and is adapted to slidably receive a mounting post to permit slidable movement of the main body over the mounting post. The mounting post has an end protruding from the main body that is pivotally connectable to the tool. An opening extends through the mounting post that is alignable with the suction passage. In operation, the main body of the switch is manually movable between a default position in which the suction passage and the opening are misaligned to block suction and an operating position in which the suction passage and opening are aligned to permit suction.