Generation of induced pluripotent stem cells from a patient with Best Dystrophy carrying 11q12.3 (BEST1 (VMD2)) mutation

Best disease (BD), also termed Best vitelliform macular dystrophy (BVMD), is a juvenile-onset form of macular degeneration and central visual loss. In this report, we generated an induced pluripotent stem cell (iPSC) line, TVGH-iPSC-012-04, from the peripheral blood mononuclear cells of a female patient with BD by using the Sendai virus delivery system. The resulting iPSCs retained the disease-causing DNA mutation, expressed pluripotent markers and could differentiate into three germ layers. We believe that BD patient-specific iPSCs provide a powerful in vitro model for evaluating the pathological phenotypes of the disease

Construction and Commissioning of PAL-XFEL Facility

The construction of Pohang Accelerator Laboratory X-ray Free-Electron Laser (PAL-XFEL), a 0.1-nm hard X-ray free-electron laser (FEL) facility based on a 10-GeV S-band linear accelerator (LINAC), is achieved in Pohang, Korea by the end of 2016. The construction of the 1.11 km-long building was completed by the end of 2014, and the installation of the 10-GeV LINAC and undulators started in January 2015. The installation of the 10-GeV LINAC, together with the undulators and beamlines, was completed by the end of 2015. The commissioning began in April 2016, and the first lasing of the hard X-ray FEL line was achieved on 14 June 2016. The progress of the PAL-XFEL construction and its commission are reported here.11Nsciescopu

Hard X-ray free-electron laser with femtosecond-scale timing jitter

The hard X-ray free-electron laser at the Pohang Accelerator Laboratory (PAL-XFEL) in the Republic of Korea achieved saturation of a 0.144 nm free-electron laser beam on 27 November 2016, making it the third hard X-ray free-electron laser in the world, following the demonstrations of the Linac Coherent Light Source (LCLS) and the SPring-8 Angstrom Compact Free Electron Laser (SACLA). The use of electron-beam-based alignment incorporating undulator radiation spectrum analysis has allowed reliable operation of PAL-XFEL with unprecedented temporal stability and dispersion-free orbits. In particular, a timing jitter of just 20 fs for the free-electron laser photon beam is consistently achieved due to the use of a state-of-the-art design of the electron linear accelerator and electron-beam-based alignment. The low timing jitter of the electron beam makes it possible to observe Bi(111) phonon dynamics without the need for timing-jitter correction, indicating that PAL-XFEL will be an extremely useful tool for hard X-ray time-resolved experiments.1143Nsciescopu