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Follow 4 young orphans who deal with their grief by forming an over-the-top glam rock band. As they rocket to stardom, their emotional adventure takes them through worlds unlike anything you've seen before.

Bietti’s crystalline dystrophy (BCD) is an intractable and progressive chorioretinal degenerative disease caused by mutations in the CYP4V2 gene, resulting in blindness in most patients. Although we and others have shown that retinal pigment epithelium (RPE) cells are primarily impaired in patients with BCD, the underlying mechanisms of RPE cell damage are still unclear because we lack access to appropriate disease models and to lesion-affected cells from patients with BCD. Here, we generated human RPE cells from induced pluripotent stem cells (iPSCs) derived from patients with BCD carrying a CYP4V2 mutation and successfully established an in vitro model of BCD, i.e., BCD patient-specific iPSC-RPE cells. In this model, RPE cells showed degenerative changes of vacuolated cytoplasm similar to those in postmortem specimens from patients with BCD. BCD iPSC-RPE cells exhibited lysosomal dysfunction and impairment of autophagy flux, followed by cell death. Lipidomic analyses revealed the accumulation of glucosylceramide and free cholesterol in BCD-affected cells. Notably, we found that reducing free cholesterol by cyclodextrins or δ-tocopherol in RPE cells rescued BCD phenotypes, whereas glucosylceramide reduction did not affect the BCD phenotype. Our data provide evidence that reducing intracellular free cholesterol may have therapeutic efficacy in patients with BCD.

Background The Response Evaluation Criteria in Solid Tumors (RECIST) was revised in 2009, based on a large dataset of 6512 patients from 16 trials. However, no gastric cancer patients were included in those data. The purpose of this study was to clarify the difference between RECIST version 1.0 and version 1.1 in advanced gastric cancer. Methods From 2004 to 2009, 129 consecutive patients with advanced gastric cancer received S-1 plus cisplatin as first-line treatment at the National Cancer Center Hospital East. Ninety-seven of these patients who had had baseline and post-treatment computed tomography scans performed were included in this study. Measurements of tumors were conducted retrospectively. Results At the baseline of first-line chemotherapy, 172 lymph nodes in 54 patients were considered to be candidate target lesions by RECIST version 1.0. However, only 38 % of the lymph nodes were classified as target lesions by RECIST version 1.1, with 47 % classified as non-target lesions and 15 % classified as non-pathological. By RECIST version 1.0, the proportion of patients with target lesions at the baseline of first-line chemotherapy was 67 % (65/97), and this percentage was significantly reduced according to RECIST version 1.1 (53 %; 51/97) (McNemar’s exact test, P  < 0.001). The findings at the baseline of second-line chemotherapy were similar (reduced from 62 to 49 %; McNemar’s exact test, P  = 0.002). Overall response rates of first-line chemotherapy were 52 % (34/65) according to RECIST version 1.0 and 55 % (28/51) according to version 1.1. Conclusions The revision of RECIST significantly reduced the proportion of patients classified with target lesions at the baselines of first-line and second-line chemotherapies. No obvious difference in overall response rates was observed.

Purpose To study the circumpapillary retinal nerve fiber layer thickness (cpRNFLT) in a 10°-wide sector using spectral-domain optical coherence tomography for diagnostic ability in early stage open-angle glaucoma (OAG). Methods cpRNFLT measurements (3.4-mm diameter centered on the disc) were obtained from 89 eyes with early stage OAG (mean deviation, −2.5 ± 1.8 decibels) and 89 age-matched normal eyes. The ability of 180°-, 90°-, 30°-, and 10°-wide cpRNFLT sectors in discriminating early stage OAG eyes was evaluated by calculating the area under the receiver-operating characteristic curves (AUCs) and sensitivity/specificity per diagnostic criteria with varying sector widths, normative data-based cpRNFLT cutoff levels and numbers of abnormal sectors. The intra- and inter-visit reproducibilities of the cpRNFLT 10°-sector measurements were studied in a separate group of eyes with normal and early stage OAG. Results The greatest AUC obtained using the cpRNFLT 10° sector, 0.924 (confidence interval, 0.875–0.958), did not differ significantly from those obtained with the 180°, 90°, and 30° sectors. Only calculations using the 10° sectors had sensitivities and specificities of 0.90 or higher with the best performance of the sensitivity/specificity of 0.92/0.94. These values tended to be better ( P  = 0.070) than with the 30° sectors, 0.85/0.94, which were also selected with several combinations of various cutoff levels and the number of abnormal sectors. The coefficients of variation for the intra- and inter-visit reproducibility of the 10°-sector measurements were 10 % or less in 32 and 24 of the 36 sectors. Conclusion The cpRNFLT 10°-sector measurements showed reasonable reproducibility and identified eyes with early stage OAG with a sensitivity and specificity of 0.92/0.94.

We axiomatize the Kalai-Smorodinsky solution (1975) in the Nash bargaining problems if the feasible sets can be finite. We show that the Kalai-Smorodinsky solution is the unique solution satisfying Continuity (in the Hausdorff topology endowed with payoffs space), Independence (which is weaker than Nash's one and essentially equivalent to Roth (1977)'s one), Symmetry, Invariance (both of which are the same as in Kalai and Smorodinsky), and Monotonicity (which reduces to a little bit weaker version of the original if the feasible sets are convex).

ObjectiveA radial artery (RA) graft is frequently used for coronary artery bypass grafting (CABG), but little information exists regarding the early- and mid-term patency associated with the harvesting procedure. The objective of this study is to compare the early- and midterm patency of the RA graft obtained using non-skeletonized and skeletonized harvesting.MethodsAltogether, 131 patients and 159 anastomoses were studied. In 85 patients the RA was harvested non-skeletonized (group A: procedures between September 2000 and November 2002), whereas in 46 patients the RA was harvested skeletonized (group B: procedures between November 2002 and April 2004). Angiography results were analyzed before discharge [A: postoperative day (POD) 14.7 ± 2.9, 75 patients, 90 anastomoses; B: POD 13.7 ± 1.9, 38 patients, 47 anastomoses], and after 1 year (A: POD 386.8 ± 149.3, 44 patients, 51 anastomoses; B: POD 267.1 ± 148.7, 11 patients, 13 anastomoses).ResultsThere was no difference in patency between the two groups (group A vs group B, 96.7% vs 100%, P = not significant [NS], in the early-term, 96.2% vs 100%, P = NS, in the mid-term). However, the perfect patency rates for groups A and B were 86.7% and 98.1%, respectively, in the early-term (P = 0.034) and 77.5% and 100%, respectively, in the mid-term (P = 0.048). The location and severity of the target vessel did not influence the angiographic results.ConclusionThe early- and mid-term patency of RA grafts was excellent, and skeletonized harvesting improved the perfect patency rates at both time points.

Background: Extracellular matrix degradation may play an important role in left ventricular (LV) remodeling. It has been reported that matrix metalloproteinase-2 (MMP-2) is activated under mechanical stress conditions. Therefore, we examined the release of MMP-2, its inhibitor and interleukin-6 (IL-6), which affects MMPs, in patients undergoing cardiac surgery with cardiopulmonary bypass (CPB). Methods: Arterial blood samples were obtained from 20 patients undergoing cardiac surgery and six patients with descending aortic replacement (as noncardiac control) with CPB. Samples were assayed for plasma MMP-2, tissue inhibitors of matrix metalloproteinase-2 (TIMP-2) and IL-6 concentration. Results: Plasma MMP-2 concentrations in the valvular disease patients were greater than in other patients ( p < 0.05) and correlated with the LV mass (r= 0.810, p < 0.0001) prior to the operation. Plasma MMP-2 concentrations decreased during CPB and gradually recovered to the baseline levels after CPB. Plasma TIMP-2 concentrations increased significantly during and after CPB in a biphasic manner. Plasma IL-6 concentrations also increased significantly during CPB ( p < 0.05 versus baseline levels). Conclusion: Plasma MMP-2 concentrations reflect the state of the left ventricle, and changes in plasma MMP-2 and TIMP-2 concentrations during CPB may play an important role in LV remodeling after cardiac surgery.

TSUBAME is a micro-satellite that the students of Tokyo Institute of Technology took the lead to develop for measuring hard X-ray polarization of Gamma-Ray Bursts(GRBs) in order to reveal the nature of the central engine of GRBs. TSUBAME has two instruments: Wide-field Burst Monitor (WBM) and Hard X-ray Compton Polarimeter (HXCP). We aim to start observing with HXCP in 15 seconds by pointing the spacecraft using Control Moment Gyro. In August 2014, we assembled TSUBAME and performed an integration test during ~2 weeks.TSUBAME by communication tests with Cute-1.7+APDII in orbit. On Nov 6 2014, TSUBAME was launched from Russia and it was put into Sun-synchronous orbit at 500 km above the ground. However, serious trouble occurred to the ham radio equipment. Therefore we could not start up the X-ray sensors until Feb 10 2015. In this paper, we report the system of TSUBAME and the progress after the launch.