\u3cem\u3eRPGRIP1\u3c/em\u3e and Cone-Rod Dystrophy in Dogs

Cone–rod dystrophies (crd) represent a group of progressive inherited blinding diseases characterized by primary dysfunction and loss of cone photoreceptors accompanying or preceding rod death. Recessive crd type 1 was described in dogs associated with an RPGRIP1 exon 2 mutation, but with lack of complete concordance between genotype and phenotype. This review highlights role of the RPGRIP1, a component of complex protein networks, and its function in the primary cilium, and discusses the potential mechanisms of genotype–phenotype discordance observed in dogs with the RPGRIP1 mutation

Polymer Pipes for Distributing Mixtures of Hydrogen and Natural Gas: Evolution of their Transport and Mechanical Properties after an Ageing under an Hydrogen Environment

International audienc

Experimental testing of the sorption-mechanical coupled behaviour of polyethylene into pressurized hydrogen

Coupling between pressurized gas sorption and tensile behavior was studied in a polyethylene (PE). Tensile and creep tests into hydrogen (30 bars) and nitrogen (30 bars) atmosphere were compared to experiments into atmospheric air. Gaseous environment was shown to have noticeable influence neither on the modulus / yield stress in tension, nor on the long term creep behavior. Mechanical consequence of a long term aging into hydrogen atmosphere was also investigated in samples previously exposed to hydrogen at various aging conditions (temperature, pressure, duration). No deleterious effect on the tensile properties of PE was evidenced