Abstract: Epidermolysis bullosa (EB) is caused by mutations in the COL7A1 gene encoding type VII collagen. EB comprises a phenotypically diverse group of inherited blistering diseases that affect the skin, and possibly mucous membranes and other organs A number of therapeutic strategies have been explored for managing the treatment of EB, such as (i) surgery, (ii) chemotherapy, (iii) use of collagenase activity inhibitors, (iv) use of antibiotics, (v) use of anti-inflammatory compound, (vi) cell therapies, (viii) protein therapy as well as (ix) gene therapy. Gene therapy of EB aimed to provide therapeutic benefit through manipulation of DNA or RNA. Anti-sense therapy targeting specific exons have been described, such as targeting exons 73 and 80. However, this kind of therapeutic strategy had to be improved as regards its pharmaceutical compliance.

Abstract: The invention relates to novel transparent glasses, vitroceramics, and transparent or translucent ceramics containing, in relation to the total composition of the glass, vitroceramic, or ceramic, at least 60 wt % of a composition having the following formula (I): (M1O)x(M2O)y(M3)2O3)z(Al2O3)100?x?y?z (I), where M1 is an element selected from among Ba and/or Sr, M2 is an element selected from among Mg or Ca, x and y are numbers such that 30?x+y?80, y is between 0% and 10% of x, M3 is an element selected from among B, Ga, or In, and z is a number between 0% and 10% of (100?x?y). The invention also relates to the method for manufacturing said compositions and to the uses of said compositions in the field of optics.

Abstract: The invention relates to novel transparent glasses, vitroceramics, and transparent or translucent ceramics containing, in relation to the total composition of the glass, vitroceramic, or ceramic, at least 60 wt% of a composition having the following formula (I): (M1O)x(M2O)y(M3)2O3)z(Al2O3)100?x?y?z (I), where M1 is an element selected from among Ba and/or Sr, M2 is an element selected from among Mg or Ca, x and y are numbers such that 30?x+y?80, y is between 0% and 10% of x, M3 is an element selected from among B, Ga, or In, and z is a number between 0% and 10% of (100?x?y). The invention also relates to the method for manufacturing said compositions and to the uses of said compositions in the field of optics.

Abstract: Inside a ducted fan turbine engine, especially one with a high or very high bypass dilution ratio, access to the gas generator compartment of the engine is effected by means of two articulated caps (34) with a C-shaped section. The upper edge of each cap (34) is connected to the fixed structure of the jet engine by means of a series of articulated arms (36) and a telescopic link (54) is inserted between the housing of the engine and the bottom of each cap. In a first stage (arrow F1), this mechanism enables the caps (34) to be brought beyond the rear edge of the outer ring (12) of the jet engine, the links (54) being locked in a compressed position. In a second stage, the caps (34) uncoupled from the ring (12) are able to pivot from the desired angle (arrow F2) around an axis (y-y') parallel to the axis (x-x') of the ducted fan turbine engine.

Abstract: Luminophors which luminesce in the blue region of the spectrum have the formula:M.sub.5 (1-.alpha.)Eu.sub.5.alpha..sup.2+ SiO.sub.4 X.sub.6wherein M represents Ba.sub.1-.beta. Sr.sub..beta. or Ba.sub.1-.beta. Ca.sub.62, with 0.ltoreq..beta..ltoreq.0.1 and 0.ltoreq..alpha..ltoreq.0.2, and X is Cl.sub.1-.gamma. Br.sub..gamma., with 0.ltoreq..gamma..ltoreq.1.