Abstract: The present invention establishes a molecular therapy for glycogen storage disease type Ia. The present invention provides an oligonucleotide of 15-30 bases comprising a nucleotide sequence complementary to die cDNA of G6PC gene with c.648G>T mutation, wherein the oligonucleotide comprises a sequence complementary to a region comprising any site between the 82nd to the 92nd nucleotide from the 5? end of exon 5 of the G6PC gene with c.648C>T mutation, a pharmacologically acceptable salt or solvate thereof. Also provided is a pharmaceutical drug comprising the oligonucleotide, a pharmacologically acceptable salt or solvate thereof (e.g., therapeutic drug for glycogen storage disease type Ia).

Abstract: A parking mechanism includes an actuator having a manual shaft rotating around the first axis, a flange provided on an outer periphery of the manual shaft, a cam rod having a coupling portion coupled to the flange and a rod body moveable along a second axis orthogonal to the first axis with a movement of the flange, a coil spring attached to the rod body, a cam that is attached to the rod body and moves along the second axis by motion of the rod body transmitting via the coil spring, a pawl member having a meshing portion and operating with a movement of the cam, and a parking gear having a tooth portion with which the meshing portion meshes. The self-holding torque of the actuator is larger than the torque applied to the manual shaft at the time of maximum compression of the coil spring.

Abstract: A cured product that is reduced in warping and has high hardness can be obtained from an active energy ray-curable resin composition which uses a methacrylic acid ester polymer that has an acid value of 50 mg KOH/g or less and a transition metal content of 1 ppm or less, while containing 80% by mole or more of a polymer that has a double bond-terminated structure represented by formula (1). (In formula (1), R represents an alkyl group, a cycloalkyl group or an aryl group.

Abstract: A magnetic component unit in the present disclosure includes: a coil component; a heat dissipator which has an engagement fixing portion and a press fixing portion; and a magnetic core fixing portion. The magnetic core fixing portion includes: a holding portion that presses against the coil component; an engaging portion that extends from a first end part of the holding portion toward the heat dissipator and has a turned-back end part; and a pressing portion that extends from a second end part of the holding portion toward the heat dissipator and has an end part bent into an L-shape. The engagement fixing portion has a U-shape, and one end and the other end of the engagement fixing portion are connected to a peripheral edge portion of the heat dissipator. The press fixing portion has a columnar shape and is provided vertically protruding from the heat dissipator.

Abstract: A magnetic component unit in the present disclosure includes: a coil component; a heat dissipator which has an engagement fixing portion and a press fixing portion; and a magnetic core fixing portion. The magnetic core fixing portion includes: a holding portion that presses against the coil component; an engaging portion that extends from a first end part of the holding portion toward the heat dissipator and has a turned-back end part; and a pressing portion that extends from a second end part of the holding portion toward the heat dissipator and has an end part bent into an L-shape. The engagement fixing portion has a U-shape, and one end and the other end of the engagement fixing portion are connected to a peripheral edge portion of the heat dissipator. The press fixing portion has a columnar shape and is provided vertically protruding from the heat dissipator.

Abstract: There is provided a method capable of producing a methacrylic-based polymer excellent in pyrolysis resistance and excellent in fabricability in a high productivity. The method for producing a methacrylic-based polymer includes the following steps (1) and (2): step (1) of using a complete mixing reactor (A) to thereby obtain the first syrup, including specific steps; step (2) of using a reaction apparatus (D) including a plug flow mixer (B) and a plug flow reactor (C) to thereby obtain the second syrup, including specific steps.

Abstract: A cured product that is reduced in warping and has high hardness can be obtained from an active energy ray-curable resin composition which uses a methacrylic acid ester polymer that has an acid value of 50 mg KOH/g or less and a transition metal content of 1 ppm or less, while containing 80% by mole or more of a polymer that has a double bond-terminated structure represented by formula (1). (In formula (1), R represents an alkyl group, a cycloalkyl group or an aryl group.

Abstract: Disclosed is an apparatus for producing a high-quality methacrylic polymer with good productivity, comprising a complete mixing type reactor 11, tubular reactors 12 and 13 which have been serially connected, and a volatile removing instrument 14, wherein at least two of the tubular reactors 12 and 13 are connected via a cooler 15 for cooling the reaction mixture. It is preferable that the cooler 15 is a multitubular cooler and the tubular reactors 12 and 13 are plug flow reactors.

Abstract: There is provided a method capable of producing a methacrylic-based polymer excellent in pyrolysis resistance and excellent in fabricability in a high productivity. The method for producing a methacrylic-based polymer includes the following steps (1) and (2): step (1) of using a complete mixing reactor (A) to thereby obtain the first syrup, including specific steps; step (2) of using a reaction apparatus (D) including a plug flow mixer (B) and a plug flow reactor (C) to thereby obtain the second syrup, including specific steps.

Abstract: A plastic optical fiber cable includes: a bare plastic optical fiber including a core formed of a polymer containing a methyl methacrylate unit, and a clad layer including a layer formed of a specific fluorine-containing olefin resin at least in the outermost layer; and a coating layer around the bare plastic optical fiber, the coating layer including a light-shielding coating layer formed of a specific nylon resin composition, a functional coating layer (C) and a functional coating layer (D) in this order, where one of the functional coating layer (C) and the functional coating layer (D) is formed of a resin composition (I) containing a polybutylene terephthalate resin as a major component or an ethylene-vinyl alcohol copolymer as a major component; the other is formed of a specific nylon resin composition (II); and a ratio of the thickness of the functional coating layer (C) to the thickness of the functional coating layer (D) is set to fall within a specific range.

Abstract: Disclosed is an apparatus for producing a high-quality methacrylic polymer with good productivity, comprising a complete mixing type reactor 11, tubular reactors 12 and 13 which have been serially connected, and a volatile removing instrument 14, wherein at least two of the tubular reactors 12 and 13 are connected via a cooler 15 for cooling the reaction mixture. It is preferable that the cooler 15 is a multitubular cooler and the tubular reactors 12 and 13 are plug flow reactors.

Abstract: A plastic optical fiber cable includes: a bare plastic optical fiber including a core formed of a polymer containing a methyl methacrylate unit, and a clad layer including a layer formed of a specific fluorine-containing olefin resin at least in the outermost layer; and a coating layer around the bare plastic optical fiber, the coating layer including a light-shielding coating layer formed of a specific nylon resin composition, a functional coating layer (C) and a functional coating layer (D) in this order, where one of the functional coating layer (C) and the functional coating layer (D) is formed of a resin composition (I) containing a polybutylene terephthalate resin as a major component or an ethylene-vinyl alcohol copolymer as a major component; the other is formed of a specific nylon resin composition (II); and a ratio of the thickness of the functional coating layer (C) to the thickness of the functional coating layer (D) is set to fall within a specific range.

Abstract: In a bidirectional power supply device, a DC-DC converter is connected such that the longer an ON time of the first switch becomes, the higher a voltage of a second positive terminal and a second negative terminal becomes. When stopping the supply of power from a first positive terminal and a first negative terminal to a second positive terminal and a second negative terminal, a control circuit turns OFF a third switch, and then operates a switching signal generation circuit so that the ON time of the first switch becomes a maximum. The switching signal generation circuit is operated so that the ON time of the first switch becomes a maximum with the third switch turned OFF in start-up when supplying power from the second positive terminal and the second negative terminal to the first positive terminal and the first negative terminal.

Abstract: The power supply unit has a sample-and-hold circuit that samples a voltage supplied from a power source to a load according to a sample timing signal and holds the voltage as an output set value for the DC/DC converter. The unit supplies a load with an output from the DC/DC converter controlled so that an output voltage of a power source becomes an output set value of the DC/DC converter when the voltage of the power source drops. Since a voltage corresponding to the voltage of the power source before the voltage drop is set to the output set value of the DC/DC converter, a stable power supply unit is obtained in which the difference is always small between the voltage of the power source in normal times and the output voltage from the DC/DC converter when the voltage of the power source temporarily fluctuates.

Abstract: A power supply device of the present invention includes a switching element and a switch that operate so that when a primary DC voltage source of the device is in a voltage drop state, the power of a secondary auxiliary voltage source thereof is mixed with the power of the DC voltage source in a balanced manner and supplied to a load. This structure can reduce the maxim amount of power storage necessary for the auxiliary voltage source and thus the number of power storage elements necessary for the auxiliary voltage source. Thus, a smaller power supply device can be provided.

Abstract: The object of the present invention is to provide a bi-directional DC-DC converter having a simplified circuit configuration. Each output of high voltage detection circuit (12) and low voltage detection circuit (15) is inputted to high-voltage side error amplifier circuit (50) and low-voltage side error amplifier circuit (51) independently disposed, which are configured so as to be inverse in polarity, and the output of high-voltage side error amplifier circuit (50) or the output of low-voltage side error amplifier circuit (51) is selected by converting direction switching circuit (22), and the output is inputted to control circuit (25) from PWM comparison circuit (21) being in common with respect to voltage step-up and decreasing operations, thereby driving the first switching element (5) and the second switching element (10).

Abstract: A DC-DC converter includes a control circuit which can change the target voltage of the reference voltage source. At the start-up of the DC-DC converter, the PWM control circuit controls so that the first switching element gradually increases the on-off ratio from a minimum on-off ratio; that the second switching element is placed in the OFF state, and that the target voltage is set higher than the normal operating voltage. When the start-up operation is completed, the PWM control circuit controls so that the second switching element starts to turn on and off, and that the target voltage is returned to the normal operating voltage.

Abstract: A limit value of an output current of power converter is defined as a first current value when a voltage at both ends of capacitor is higher than a voltage at both ends of battery, and a limit value of the output current thereof is defined as a second current value when the voltages are substantially equal to each other, the first current value is set to be smaller than the second current value. Thus, when a voltage at both ends of capacitor and a voltage at both ends of battery are substantially equal, heat is hardly generated. The limit value of the output current is set to a maximum current consumed by load. When a voltage at both ends of capacitor is higher than a voltage at both ends of battery, the limit value of the output current is set to be smaller than the second current value, and thereby heat generation is suppressed. Thus, the size of the heat dissipating components can be reduced, and a power supply system whose entire size can be minimized can be provided.