Rituximab therapy in nephrotic syndrome due to AH amyloidosis

This is an electronic version of an article published in Amyloid 2009, Vol. 16, No. 3 : Pages 178-180. Amyloid is available online at: http://informahealthcare.com/doi/pdf/10.1080/13506120903090940We report a patient with AH amyloidosis associated with lymphoplasmacytic leukemia that has remained in a stable state with a nephrotic syndrome for 17 months since the commencement of cyclic rituximab therapy aimed at suppression of pathogenetic gamma heavy chains. Free light chains in serum and CD20-positive cells in peripheral blood were useful as hematological markers in the patient. Rituximab might be a potent therapeutic option for AH amyloidosis associated with a B-cell lymphoproliferative disorder.ArticleAMYLOID. 16(3):178-180 (2009)journal articl

Differential gene expression in the murine gastric fundus lacking interstitial cells of Cajal.

BACKGROUND: The muscle layers of murine gastric fundus have no interstitial cells of Cajal at the level of the myenteric plexus and only possess intramuscular interstitial cells and this tissue does not generate electric slow waves. The absence of intramuscular interstitial cells in W/WV mutants provides a unique opportunity to study the molecular changes that are associated with the loss of these intercalating cells. METHOD: The gene expression profile of the gastric fundus of wild type and W/WV mice was assayed by murine microarray analysis displaying a total of 8734 elements. Queried genes from the microarray analysis were confirmed by semi-quantitative reverse transcription-polymerase chain reaction. RESULTS: Twenty-one genes were differentially expressed in wild type and W/WV mice. Eleven transcripts had 2.0-2.5 fold higher mRNA expression in W/WV gastric fundus when compared to wild type tissues. Ten transcripts had 2.1-3.9 fold lower expression in W/WV mutants in comparison with wild type animals. None of these genes have ever been implicated in any bowel motility function. CONCLUSIONS: These data provides evidence that several important genes have significantly changed in the murine fundus of W/WV mutants that lack intramuscular interstitial cells of Cajal and have reduced enteric motor neurotransmission

Differential gene expression profile in the small intestines of mice lacking pacemaker interstitial cells of Cajal.

BACKGROUND: We previously identified eight known and novel genes differentially expressed in the small intestines of wild type and W/WV mice, which have greatly reduced populations of the interstitial cells of Cajal, that are responsible for the generation of electrical slow waves, by using a differential gene display method. METHODS: By using the same method we isolated additional candidate genes that were specifically down- or up-regulated in W/WV mice. Novel transcripts were designated as DDWMEST. RESULTS: We isolated seven candidates that were specifically down- or up-regulated in W/WV mice. Two novel transcripts, DDWMEST 1 and -91 were increased in both fed and fasted W/WV mice. Expression of another five genes was suppressed in W/WV mice: ARG2 (Arginase II), ONZIN (encoding leukemia inhibitory factor regulated protein), and three novel transcripts: DDWMEST62, -84, and -100. Together with the previous report, we identified fifteen differentially expressed genes in total in the small intestines of W/WV mice. Eight of these genes were reduced in the jejunums of W/WV mice compared to age matched wild type mice, whereas the other seven genes showed an increase in expression. Differential expression was the same in fasted and fed animals, suggesting that the differences were independent of the dietetic state of the animal. CONCLUSIONS: Several known and novel genes are differentially expressed in the small intestines of W/WV mice. Differential gene comparison might contribute to our understanding of motility disorders associated with the loss of the interstitial cells of Cajal.RIGHTS : This article is licensed under the BioMed Central licence at http://www.biomedcentral.com/about/license which is similar to the 'Creative Commons Attribution Licence'. In brief you may : copy, distribute, and display the work; make derivative works; or make commercial use of the work - under the following conditions: the original author must be given credit; for any reuse or distribution, it must be made clear to others what the license terms of this work are

A 100 W-Class Water-Vapor Hall Thruster for Constellations and Space Explorations by SmallSats

The laboratory models of a water-vapor Hall thruster and LaB6 thermionic cathode were developed and tested. To optimize the thruster design to water-vapor propellant, the geometrical investigation was conducted. After testing six different models, the smallest thruster, with an outer diameter of 20 mm, was found to be the most suitable for 100-W class operation. This thruster was able to be operated less than 100 W at 200 V. In addition, the discharge power was suppressed to 200 W even at 300 V. Based on the plume diagnostics, the thrust force of 2.9 mN, specific impulse of 650 s, and anode efficiency of 4.6 % were obtained as a representative performance of this 300 V operating point. After the thruster operation was achieved, the cathode coupling test was conducted to demonstrate electron emission under water-vapor plasma existence. As a result of this experiment, the effective increase in electron current compared to the previous stand-alone tests was confirmed as well as the compatability to the water-vapor plasma plume. On the other hand, the electron emission current has not achieved 100 mA-class yet and the required heating power was predicted over 100 W; thus, further improvement is progressing

Search for Outer Massive Bodies around Transiting Planetary Systems: Candidates of Faint Stellar Companions around HAT-P-7

We present results of direct imaging observations for HAT-P-7 taken with the Subaru HiCIAO and the Calar Alto AstraLux. Since the close-in transiting planet HAT-P-7b was reported to have a highly tilted orbit, massive bodies such as giant planets, brown dwarfs, or a binary star are expected to exist in the outer region of this system. We show that there are indeed two candidates for distant faint stellar companions around HAT-P-7. We discuss possible roles played by such companions on the orbital evolution of HAT-P-7b. We conclude that as there is a third body in the system as reported by Winn et al. (2009, ApJL, 763, L99), the Kozai migration is less likely while planet-planet scattering is possible.Comment: 8 pages, 3 figures, 2 tables, PASJ in pres

Polarimetric Imaging of Large Cavity Structures in the Pre-transitional Protoplanetary Disk around PDS 70: Observations of the disk

We present high resolution H-band polarized intensity (PI; FWHM = 0."1: 14 AU) and L'-band imaging data (FWHM = 0."11: 15 AU) of the circumstellar disk around the weak-lined T Tauri star PDS 70 in Centaurus at a radial distance of 28 AU (0."2) up to 210 AU (1."5). In both images, a giant inner gap is clearly resolved for the first time, and the radius of the gap is ~70 AU. Our data show that the geometric center of the disk shifts by ~6 AU toward the minor axis. We confirm that the brown dwarf companion candidate to the north of PDS 70 is a background star based on its proper motion. As a result of SED fitting by Monte Carlo radiative transfer modeling, we infer the existence of an optically thick inner disk at a few AU. Combining our observations and modeling, we classify the disk of PDS 70 as a pre-transitional disk. Furthermore, based on the analysis of L'-band imaging data, we put an upper limit mass of companions at ~30 to ~50MJ within the gap. Taking account of the presence of the large and sharp gap, we suggest that the gap could be formed by dynamical interactions of sub-stellar companions or multiple unseen giant planets in the gap.Comment: accepted by APJ