Abstract: The present invention relates to the use of CFTR correctors in the treatment of genetic disorders affecting striated muscle selected from sarcoglycanopathies, Brody's disease (BD) and the recessive forms of Cathecolaminergic Polymorphic Ventricular Tachycardia (CPVT).

Abstract: The present invention relates to the use of CFTR correctors in the treatment of genetic disorders affecting striated muscle selected from sarcoglycanopathies, Brody's disease (BD) and the recessive forms of Cathecolaminergic Polymorphic Ventricular Tachycardia (CPVT).

Abstract: The present invention relates to the use of CFTR correctors in the treatment of genetic disorders affecting striated muscle selected from sarcoglycanopathies, Brody's disease (BD) and the recessive forms of Cathecolaminergic Polymorphic Ventricular Tachycardia (CPVT).

Abstract: Devices for selective hydrogenation of azide and peroxide compounds include at least one porous element, for example a solid metallic structure positioned within a reaction tube of a tubular housing. The porous element includes a framework of interconnected elements establishing a plurality of shells respectively defining a plurality of hollow spaces of predetermined geometrical configuration. Each of the shells includes holes that fluid-connect adjacent ones of the spaces to thereby allow a cross-flow of the azide or peroxide compounds through the porous element.

Abstract: The present invention relates to a device for treatment of material transported through the device comprising at least one porous element consisting of solid, for example metallic, structure which allows cross-flow of the material through the porous element. A device in accordance with the invention is particularly useful as mixer or heat exchanger or to carry out chemical reactions under homogenous and heterogeneous conditions. Such a device hereinafter also referred as reactor may comprises a tube (1) having a cylindrical wall (2) with one inlet end (3) and one outlet end (4). Arranged in the tube (1) is at least one cylindrical porous element (5) consisting of solid metal structure, wherein said porous element (5) comprises a plurality of hollow spaces that are connected to each other and form an interconnected cavity network and wherein the at least one porous element (5) and the cylindrical wall (2) are made in one piece. The porosity ? of the at least one porous element (5) is between 0.8 and 0.95.

Abstract: The present invention relates to a device for treatment of material transported through the device comprising at least one porous element consisting of solid, for example metallic, structure which allows cross-flow of the material through the porous element. A device in accordance with the invention is particularly useful as mixer or heat exchanger or to carry out chemical reactions under homogenous and heterogeneous conditions. Such a device hereinafter also referred as reactor may comprises a tube (1) having a cylindrical wall (2) with one inlet end (3) and one outlet end (4). Arranged in the tube (1) is at least one cylindrical porous element (5) consisting of solid metal structure, wherein said porous element (5) comprises a plurality of hollow spaces that are connected to each other and form an interconnected cavity network and wherein the at least one porous element (5) and the cylindrical wall (2) are made in one piece. The porosity ? of the at least one porous element (5) is between 0.8 and 0.95.

Abstract: The present invention relates to a device for treatment of material transported through the device comprising at least one porous element consisting of solid, for example metallic, structure which allows cross-flow of the material through the porous element. The invention also relates to various types of uses of the device. A device in accordance with the invention is particularly useful to carry out chemical reactions under homogenous and heterogeneous conditions. Such a device hereinafter also referred as reactor may comprises a tube (1) having a cylindrical wall (2) with one inlet end (3) and one outlet end (4). Arranged in the tube (1) is at least one cylindrical porous element (5) consisting of solid metal structure, wherein said porous element (5) comprises a plurality of hollow spaces that are connected to each other and form an interconnected cavity network and wherein the at least one porous element (5) and the cylindrical wall (2) are made in one piece.