Abstract: The present invention relates to a use of a novel compound, for preventing, improving or treating amyotrophic lateral sclerosis (ALS). The present inventors have found that SOD1 aggregation is one of the important causes of ALS, and have proposed the possibility that WT-SOD1 aggregation, caused by suppressing the regulation of intracellular stress or TDP-43, may be a cause of sALS. In addition, the present inventors have discovered the novel compound PRG-A-01 (SLC-B036) as a SOD1 aggregation and misfolding inhibitor. The compound exhibited a protective effect against muscle weakness and movement disorder in an ALS mouse model. According to the result of a histological analysis, intraspinal nerves were maintained by means of a treatment using PRG-A-01 (SLC-B036). In addition, the present inventors have obtained a candidate compound (PRG-A-04) which can be a more optimized drug. Consequently, the compound of the present invention may be usefully employed in developing a therapeutic agent for ALS.

Abstract: The present invention relates to a use of a novel compound, for preventing, improving or treating amyotrophic lateral sclerosis (ALS), wherein the present inventors have found that SOD1 aggregation is one of the important causes of ALS, and have proposed the possibility that WT-SOD1 aggregation, caused by suppressing the regulation of intracellular stress or TDP-43, may be a cause of sALS. In addition, the present inventors have discovered the novel compound PRG-A-01(SLC-B036) as a SOD1 aggregation and misfolding inhibitor.

Abstract: The present invention relates to a novel method for synthesizing a decursin derivative, the method comprising: a step (I) for preparing a solution by mixing cinnamyl bromide and an N-Methyl-2-pyrrolidone [N-Methyl-2-pyrrolidone (NMP)] solvent; a step (II) for preparing a solution by mixing decursinol, a tetrahydrofuran (THF) solvent, and sodium hydride (NaH); and a step (III) for obtaining a decursin derivative by mixing the solutions prepared in step (I) and step (II). The method can increase the yield of the obtained decursin derivative compound and enables mass production.

Abstract: The present invention relates to granules comprising Progerinin, capsules or sachets using same, and a preparation method therefor. More specifically, in order to develop a formulation that is advantageous when applying, to patients, Progerinin, which is a poorly water-soluble drug having a Hutchinson-Gilford progeria treatment effect, Progerinin granules comprising a specific water-soluble polymer, solubility enhancer and excipient at an optimum composition ratio have been developed and oral solid preparations such as capsules or sachets have been developed using the granules, such that dispersion stability and bioavailability of Progerinin can be improved and storage, transport and patient drug compliance can also be improved.

Abstract: The present invention relates to a nanosuspension formulation suitable for a progerinin drug, which is insoluble, and a preparation method therefor, wherein the progerinin nanosuspension is a vehicle composition for improving dispersive stability of a progerinin drug(a), which is insoluble, and employs hydroxypropylmethyl cellulose (HPMC)(b) or hydroxypropyl cellulose (HPC)(b?), TPGS(c) or sodium dioctyl succinate (DOSS)(c?), and potassium sorbate(d), and a mixture thereof was subjected to wet-type ball milling in a Dyno-Mill chamber to prepare a white nanosuspension containing a progerinin drug with an average particle diameter of 100 nm to 300 nm.

Abstract: The present invention relates to a novel method for synthesizing a decursin derivative, enabling high-yield mass synthesis of a decursin derivative (PRG-A-04) represented by compound I through a reaction between decursinol and an intermediate compound in which a Boc protecting group is introduced to an OH group of 3-(3-methoxy-4-nitrophenyl)prop-2-en-1-ol in the presence of a palladium catalyst and xantphos.

Abstract: The present invention relates to a composition for prevention or treatment of neurofibromatosis type 2 syndrome, wherein in contrast to the conventional T?R1 kinase inhibitor TEW7197, the compound represented by chemical formula 1, a pharmaceutically acceptable salt thereof, a solvate thereof, a stereoisomer thereof, or a combination thereof according to the present invention suppresses TGF-? receptor 1 (T?R1)-mediated RKIP reduction while not inhibiting normal TGF-? signaling and thus, can be used as a novel form of a therapeutic agent for neurofibromatosis type 2 syndrome, which can solve the side effect problem caused by the inhibition of normal TGF-? signaling.

Abstract: The present invention relates to a novel method for synthesizing a decursin derivative, the method comprising: a step (I) for preparing a solution by mixing cinnamyl bromide and an N-Methyl-2-pyrrolidone [N-Methyl-2-pyrrolidone (NMP)] solvent; a step (II) for preparing a solution by mixing decursinol, a tetrahydrofuran (THF) solvent, and sodium hydride (NaH); and a step (III) for obtaining a decursin derivative by mixing the solutions prepared in step (I) and step (II). The method can increase the yield of the obtained decursin derivative compound and enables mass production.

Abstract: The present invention relates to a use of a novel compound, for preventing, improving or treating amyotrophic lateral sclerosis (ALS). The present inventors have found that SOD1 aggregation is one of the important causes of ALS, and have proposed the possibility that WT-SOD1 aggregation, caused by suppressing the regulation of intracellular stress or TDP-43, may be a cause of sALS. In addition, the present inventors have discovered the novel compound PRG-A-01(SLC-B036) as a SOD1 aggregation and misfolding inhibitor. The compound exhibited a protective effect against muscle weakness and movement disorder in an ALS mouse model. According to the result of a histological analysis, intraspinal nerves were maintained by means of a treatment using PRG-A-01(SLC-B036). In addition, the present inventors have obtained a candidate compound (PRG-A-04) which can be a more optimized drug. Consequently, the compound of the present invention may be usefully employed in developing a therapeutic agent for ALS.

Abstract: A composition for preventing or treating an aging-related disease includes a novel decursin derivative as an active ingredient, wherein the novel decursin derivative has exhibited an excellent effect of inhibiting progerin expression and excellent effect of inhibiting binding between progerin and lamin A, and it has been confirmed that the novel decursin derivative prolongs the survival period of animal models in which progerin was induced.