Rpgrip1 is required for rod outer segment development and ciliary protein trafficking in zebrafish

The authors would like to thank the Royal Society of London, the National Eye Research Centre, the Visual Research Trust, Fight for Sight, the W.H. Ross Foundation, the Rosetrees Trust, and the Glasgow Children’s Hospital Charity for supporting this work. This work was also supported by the Deanship of Scientific Research at King Saud University for funding this research (Research Project) grant number ‘RGP – VPP – 219’.Mutations in the RPGR-interacting protein 1 (RPGRIP1) gene cause recessive Leber congenital amaurosis (LCA), juvenile retinitis pigmentosa (RP) and cone-rod dystrophy. RPGRIP1 interacts with other retinal disease-causing proteins and has been proposed to have a role in ciliary protein transport; however, its function remains elusive. Here, we describe a new zebrafish model carrying a nonsense mutation in the rpgrip1 gene. Rpgrip1homozygous mutants do not form rod outer segments and display mislocalization of rhodopsin, suggesting a role for RPGRIP1 in rhodopsin-bearing vesicle trafficking. Furthermore, Rab8, the key regulator of rhodopsin ciliary trafficking, was mislocalized in photoreceptor cells of rpgrip1 mutants. The degeneration of rod cells is early onset, followed by the death of cone cells. These phenotypes are similar to that observed in LCA and juvenile RP patients. Our data indicate RPGRIP1 is necessary for rod outer segment development through regulating ciliary protein trafficking. The rpgrip1 mutant zebrafish may provide a platform for developing therapeutic treatments for RP patients.Publisher PDFPeer reviewe

Effect of temperature and hydrogen exposure on the mechanical response of filled elastomers: A study on acrylonitrile butadiene rubbers filled with carbon black

International audienceFilled elastomers are widely used in components within the gas infrastructures. Currently, it is necessary to evaluate their suitability to harsh conditions of temperature and pressure required by the hydrogen applications. The effect of the environmental conditions on the material mechanical response (up to fracture) has been evaluated, by performing tests at different temperatures or under pressurized hydrogen, on three carbon black-filled acrylonitrile butadiene rubbers. With the increase of temperature, the fracture resistance resulted to decrease and the crack advancement rate increased. If the gas pressure applied during testing increases, the fracture toughness evaluated at the onset of unstable crack propagation increases while the crack advancement rate decreases. Some effects related to the gas type (hydrogen or nitrogen) were also observed. Moreover, if the material was previously exposed to pressurized hydrogen, no effects on the mechanical response were observed

Economie des entreprises: La méthode budgétaire appliquée à la gestion des exploitations

In order to simplify administrative work and to give it maximum efficienty it is necessary to show up the various items of expenses and receipts clearly and the margins which do not comply with reasonable objectives (exceptions) and also how great the gap is (disparity). The measurement of such "disparities" and the pointing out of "exceptions" by means of a permanent budgetary control are the most frequently used management "tools"

\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

Economie des entreprises: La méthode budgétaire appliquée à la gestion des exploitations

[eng] The budgetary method applied to farm and agricultural cooperative management - In order to simplify administrative work and to give it maximum efficienty it is necessary to show up the various items of expenses and receipts clearly and the margins which do not comply with reasonable objectives (exceptions) and also how great the gap is (disparity). The measurement of such "disparities" and the pointing out of "exceptions" by means of a permanent budgetary control are the most frequently used management "tools". [fre] Pour simplifier le travail de gestion tout en lui donnant l'efficacité maximum, il faut mettre en évidence les postes (dépenses, recettes) et les marges qui ne suivent pas les objectifs « raisonnables » (exception), et indiquer de combien ils s'en écartent (écarts). La mesure des « écarts » et la mise en évidence des « exceptions » par le contrôle budgétaire permanent sont des outils de gestion de plus en plus employés.

Creep compression behaviour of a polyurethane foam from cryogenic temperatures: size effect and long-term prediction

The objective of this work was to predict the long term behavior of Polyurethane foam (PU) at very low-temperature, by applying the time-temperature superposition principle (TTSP). The experimental background of the TTSP was based on a Dynamical Mechanical Analysis technique. Two issues arise from this experimental approach: the relevance of the temperature range to apply the TTSP, and the possible size-effect associated to the small DMA samples. Firstly, on the studied temperature range (-170°C; +180°C) many transitions have been observed, particularly from -20°C. Thus to apply the TTSP, it would be necessary to limit the temperature range (between temperature of molecular transitions, i.e. from -20°C up to 80°C). At very low temperatures, DMA spectra did not evidence any viscoelastic domain. However a deformation has been measured during creep tests in the same temperature range. So it would be necessary to determine which micro-mechanism is responsible for the observed deformation. Secondly, it was important to determine if the volume of DMA sample was representative. Several techniques have shown that a representative volume would be reached between 8 and 12mm3

Creep compression behaviour of a polyurethane foam from cryogenic temperatures: size effect and long-term prediction

The objective of this work was to predict the long term behavior of Polyurethane foam (PU) at very low-temperature, by applying the time-temperature superposition principle (TTSP). The experimental background of the TTSP was based on a Dynamical Mechanical Analysis technique. Two issues arise from this experimental approach: the relevance of the temperature range to apply the TTSP, and the possible size-effect associated to the small DMA samples. Firstly, on the studied temperature range (-170°C; +180°C) many transitions have been observed, particularly from -20°C. Thus to apply the TTSP, it would be necessary to limit the temperature range (between temperature of molecular transitions, i.e. from -20°C up to 80°C). At very low temperatures, DMA spectra did not evidence any viscoelastic domain. However a deformation has been measured during creep tests in the same temperature range. So it would be necessary to determine which micro-mechanism is responsible for the observed deformation. Secondly, it was important to determine if the volume of DMA sample was representative. Several techniques have shown that a representative volume would be reached between 8 and 12mm3