Nul op de meter:hoe verder met energierenovatie

Afsluitende brochure van het RAAK-project Op naar beter, nul op de meter! Verschillende auteurs uit het werkveld hebben een bijdrage geleverd. Met een voorwoord van gedeputeerde Nienke Homan

Simple model for laser-produced, mass-limited water-droplet plasmas

Plasmas, produced by a neodymium yttrium aluminum garnet (Nd:YAG) laser pulse focused on a small water droplet and used for the generation of extreme ultraviolet light, can be described by a relatively simple model due to the fact that thermodynamic equilibrium can be assumed for the most important phase. Only three time-dependent variables¿radius, expansion speed, and internal energy¿are needed to describe the physics of the plasma. Nevertheless, it predicts quantities such as the size and the spectrum rather well. It is expected that the theory and the model presented here can also be applied to other laser-produced plasmas

Nul op de meter: hoe verder met energierenovatie

Afsluitende brochure van het RAAK-project Op naar beter, nul op de meter! Verschillende auteurs uit het werkveld hebben een bijdrage geleverd. Met een voorwoord van gedeputeerde Nienke Homan

In utero and postnatal VX-770 administration rescues multiorgan disease in a ferret model of cystic fibrosis

Cystic fibrosis (CF) is a multiorgan disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR). In patients with CF, abnormalities initiate in several organs before birth. However, the long-term impact of these in utero pathologies on disease pathophysiology is unclear. To address this issue, we generated ferrets harboring a VX-770 (ivacaftor)-responsive CFTR G551D mutation. In utero VX-770 administration provided partial protection from developmental pathologies in the pancreas, intestine, and male reproductive tract. Homozygous CFTR G551D/G551D animals showed the greatest VX-770-mediated protection from these pathologies. Sustained postnatal VX-770 administration led to improved pancreatic exocrine function, glucose tolerance, growth and survival, and to reduced mucus accumulation and bacterial infections in the lung. VX-770 withdrawal at any age reestablished disease, with the most rapid onset of morbidity occurring when withdrawal was initiated during the first 2 weeks after birth. The results suggest that CFTR is important for establishing organ function early in life. Moreover, this ferret model provides proof of concept for in utero pharmacologic correction of genetic disease and offers opportunities for understanding CF pathogenesis and improving treatment