Abstract: A metallic sealing assembly between a rotating shaft and a fixed frame comprises: a flexible metallic seal having two tori, inner and outer, that are concentric and of different average diameters, comprising a metal envelope which encases and holds the inner and outer tori; a shaft rotating about an axis of rotation, comprising an annular shoulder against which the seal comes to press; a counter-face, comprising a contact face against which the seal comes to press; a wedge block positioned around the rotating shaft such that the seal is held between the wedge block and the contact face, the wedge block mounted to slide on the rotating shaft; a metal part which forms a cap through which the rotating shaft passes and which is secured to the frame and/or to the counter-face, the rotating shaft being free to rotate with respect to the cap and axially secured to the cap.

Abstract: A static metal seal comprising an outer sealing layer, wherein the outer sealing layer comprises a textured surface that is configured to come into contact with the surfaces that are to be sealed, the textured surface comprising a network of depressions spaced apart from one another on the textured surface, wherein the depressions are blind, do not pass all the way through the outer sealing layer, and do not communicate with one another.

Abstract: A metal sealing system for a triple eccentricity butterfly valve includes a dynamic metal seal, having a metal core surrounded by an external coating; a metal case with an external surface that has an inclined conical shape, including a first housing inside which the dynamic metal seal is mounted; a metal cover fixed to the metal case to allow it to close by flush fitting of the upper or lower faces of the metal cover and the metal case, the metal case having a second housing into which the metal cover fits, the dynamic metal seal being located between the metal cover and the surface of the metal case defining the first housing.

Abstract: The main purpose of the invention is a metal sealing system (6) for a triple eccentricity butterfly valve (30) comprising: a dynamic metal seal (21), comprising a metal core surrounded by an external coating; a metal case (15) with an external surface that has an inclined conical shape, comprising a first housing inside which the dynamic metal seal (21) is mounted; a metal cover (29) fixed to the metal case (15) to allow it to close by flush fitting of the upper or lower faces of the metal cover (29) and the metal case (15), the metal case comprising a second housing into which the metal cover fits, the dynamic metal seal (21) being located between the metal cover (29) and the surface of the metal case (15) defining the first housing.

Abstract: A static metal seal comprising an outer sealing layer, wherein the outer sealing layer comprises a textured surface that is configured to come into contact with the surfaces that are to be sealed, the textured surface comprising a network of depressions spaced apart from one another on the textured surface, wherein the depressions are blind, do not pass all the way through the outer sealing layer, and do not communicate with one another.

Abstract: A metallic sealing assembly between a rotating shaft and a fixed frame comprises: a flexible metallic seal having two tori, inner and outer, that are concentric and of different average diameters, comprising a metal envelope which encases and holds the inner and outer tori; a shaft rotating about an axis of rotation, comprising an annular shoulder against which the seal comes to press; a counter-face, comprising a contact face against which the seal comes to press; a wedge block positioned around the rotating shaft such that the seal is held between the wedge block and the contact face, the wedge block mounted to slide on the rotating shaft; a metal part which forms a cap through which the rotating shaft passes and which is secured to the frame and/or to the counter-face, the rotating shaft being free to rotate with respect to the cap and axially secured to the cap.

Abstract: The present invention provides for compounds of formula (I) wherein R1, R2, R3, R4, R5, and R6 have any of the values defined in the specification, and pharmaceutically acceptable salts thereof, that are useful as agents in the treatment of diseases and conditions mediated and modulated by CFTR, including cystic fibrosis, Sjögren's syndrome, pancreatic insufficiency, chronic obstructive lung disease, and chronic obstructive airway disease. Also provided are pharmaceutical compositions comprised of one or more compounds of formula (I).

Abstract: The present invention discloses compounds according to Formula I: wherein R1, R2, and Cy are as defined herein. The present invention relates to compounds inhibiting IRAK family kinases, methods for their production, pharmaceutical compositions comprising the same, and methods of treatment using the same, for the prophylaxis and/or treatment of inflammatory diseases, autoimmune diseases and/or proliferative diseases by administering the compound of the invention.

Abstract: The present invention provides for compounds of formula (I) wherein R1, R2, R3, R4, R5, and R6 have any of the values defined in the specification, and pharmaceutically acceptable salts thereof, that are useful as agents in the treatment of diseases and conditions mediated and modulated by CFTR, including cystic fibrosis, Sjögren's syndrome, pancreatic insufficiency, chronic obstructive lung disease, and chronic obstructive airway disease. Also provided are pharmaceutical compositions comprised of one or more compounds of formula (I).

Abstract: An annular assembly for compressing a seat of a ball-valve, including: a valve body or insert, including an annular internal counter-bore that defines an inner counter-bore diameter; a sleeve inserted into the annular internal counter-bore, defining at least one outer diameter of the sleeve; the body or the insert defining with the sleeve an annular housing in which the assembly includes, in succession: a downstream stack of head rings, an annular head shim, a stack of mechanisms of compression, an annular base shim, and an upstream stack of base rings.

Abstract: The present invention provides for compounds of formula (I) wherein R1, R2, R3, R4, R5, and R6 have any of the values defined in the specification, and pharmaceutically acceptable salts thereof, that are useful as agents in the treatment of diseases and conditions mediated and modulated by CFTR, including cystic fibrosis, Sjögren's syndrome, pancreatic insufficiency, chronic obstructive lung disease, and chronic obstructive airway disease. Also provided are pharmaceutical compositions comprised of one or more compounds of formula (I).

Abstract: The present invention relates to, inter alia, treatment of muscle dystrophy (e.g., Duchenne Muscular Dystrophy), for example, using a composition, e.g., a composition comprising Compound (I), or a pharmaceutically acceptable salt, prodrug or metabolite thereof.

Abstract: The present invention provides for compounds of formula (I) wherein R1, R2, R3, R4, R5, and R6 have any of the values defined in the specification, and pharmaceutically acceptable salts thereof, that are useful as agents in the treatment of diseases and conditions mediated and modulated by CFTR, including cystic fibrosis, Sjögren's syndrome, pancreatic insufficiency, chronic obstructive lung disease, and chronic obstructive airway disease. Also provided are pharmaceutical compositions comprised of one or more compounds of formula (I).

Abstract: A corrector agent capable of stabilizing a newly synthesized cystic fibrosis transmembrane conductance regulator (CFTR) protein, useful in the treatment of cystic fibrosis.

Abstract: The present invention discloses compounds according to Formula I: wherein R1, R2, and Cy are as defined herein. The present invention relates to compounds inhibiting IRAK family kinases, methods for their production, pharmaceutical compositions comprising the same, and methods of treatment using the same, for the prophylaxis and/or treatment of inflammatory diseases, autoimmune diseases and/or proliferative diseases by administering the compound of the invention.

Abstract: The present application provides a static seal assembly for a rod configured for reciprocal motion. The static seal assembly is generally sized to fit within a conventional cup of a stuffing box. The static seal assembly has a body with a base and a balloon that fit within the cup. The balloon is made of an expandable material and is selectively in fluid communication with a pressurized fluid source. When pressurized, or inflated, the balloon expands and forms a seal interface with the outer surface of the rod.

Abstract: The present invention provides for compounds of formula (I) wherein R1, R2, R3, R4, R5, and R6 have any of the values defined in the specification, and pharmaceutically acceptable salts thereof, that are useful as agents in the treatment of diseases and conditions mediated and modulated by CFTR, including cystic fibrosis, Sjögren's syndrome, pancreatic insufficiency, chronic obstructive lung disease, and chronic obstructive airway disease. Also provided are pharmaceutical compositions comprised of one or more compounds of formula (I).

Abstract: The present invention provides for compounds of formula (I) wherein R1, R2, R3, R4, R5, and R6 have any of the values defined in the specification, and pharmaceutically acceptable salts thereof, that are useful as agents in the treatment of diseases and conditions mediated and modulated by CFTR, including cystic fibrosis, Sjögren's syndrome, pancreatic insufficiency, chronic obstructive lung disease, and chronic obstructive airway disease. Also provided are pharmaceutical compositions comprised of one or more compounds of formula (I).

Abstract: The present invention provides for compounds of formula (I) wherein R1, R2, R3, and R4 have any of the values defined in the specification, and pharmaceutically acceptable salts thereof, that are useful as agents in the treatment of diseases and conditions mediated and modulated by CFTR, including cystic fibrosis, Sjögren's syndrome, pancreatic insufficiency, chronic obstructive lung disease, and chronic obstructive airway disease. Also provided are pharmaceutical compositions comprised of one or more compounds of formula (I).

Abstract: An annular assembly for compressing a seat of a ball-valve, including: a valve body or insert, including an annular internal counter-bore that defines an inner counter-bore diameter; a sleeve inserted into the annular internal counter-bore, defining at least one outer diameter of the sleeve; the body or the insert defining with the sleeve an annular housing in which the assembly includes, in succession: a downstream stack of head rings, an annular head shim, a stack of mechanisms of compression, an annular base shim, and an upstream stack of base rings.