Gene therapy and genome editing for primary immunodeficiency diseases

In past two decades the gene therapy using genetic modified autologous hematopoietic stem cells (HSCs) transduced with the viral vector has become a promising alternative option for treating primary immunodeficiency diseases (PIDs). Despite of some pitfalls at early stage clinical trials, the field of gene therapy has advanced significantly in the last decade with improvements in viral vector safety, preparatory regime for manufacturing high quality virus, automated CD34 cell purification. Hence, the overall outcome from the clinical trials for the different PIDs has been very encouraging. In addition to the viral vector based gene therapy, the recent fast moving forward developments in genome editing using engineered nucleases in HSCs has provided a new promising platform for the treatment of PIDs. This review provides an overall outcome and progress in gene therapy clinical trials for SCID-X, ADA-SCID, WAS, X- CGD, and the recent developments in genome editing technology applied in HSCs for developing potential therapy, particular in the key studies for PIDs

Stress and its effect on optical properties of AlN nanorods

2009-2010 > Academic research: refereed > Publication in refereed journalVersion of RecordPublishe

Thermal field over Tibetan Plateau and Indian summer monsson rainfall

The interannual variability of the temperature anomalies over the Tibetan Plateau (25-45 °N, 75-105 °E) is examined in relation to the Indian summer monsoon rainfall (ISMR: June to September total rainfall). For this purpose, the temperature anomaly data of the central-eastern Tibetan Plateau is divided into three regions using principal component analysis and the ISMR data for the period 1957-89 have been used. It is found that the January temperature anomaly of Region 2 has a significant negative relationship (r = -0.67) with the ISMR of the subsequent season. This region is located over the northeastern part of the Tibetan Plateau, mostly in Qinghai province, including the Bayan Harr Mountain range and the Qaidam Basin. This relationship is consistent and robust during the period of analysis and can be used to predict the strength of the Indian summer monsoon in the subsequent season. It was found that the January temperature anomaly in this region was associated with a persistent winter circulation pattern over the Eurasian continent during January through to March. Finally, the variation patterns of the temperature anomalies in all three regions over the central-eastern Tibetan Plateau during extreme years of the ISMR are examined. It is concluded that the January temperature anomaly over the northeastern Tibetan Plateau can be useful in forecasting the drought and flood conditions over India, especially in predicting the monsoon rainfall over the areas lying along the monsoon trough

A Novel Biphasic-Current-Pulse Calibration Technique for Electrical Neural Stimulation

One of the major challenge in neural prosthetic device design is to ensure charge-balanced stimulation. This paper presents a new calibration technique to minimize the mismatch between anodic and cathodic current amplitudes. The proposed circuit mainly consists of a digital and an analog calibration, where a successive approximation register (SAR) logic and a comparator are used in digital calibration while a source follower is adopted in analog calibration. With a 0. 18 μm high voltage CMOS process, the simulation shows that the maximum current mismatch is 45 nA (<0.05%).published_or_final_versio

π Junction to probe antiphase s-Wave pairing in iron pnictide superconductors

Josephson junctions between a FeAs-based superconductor with antiphase s-wave pairing and a conventional s-wave superconductor are studied. The translational invariance in a planar junction between a single crystal pnictide and an aluminum metal greatly enhances the relative weight of electron pockets in the pnictide to the critical current. In a wide doping region of the pnictide, a planar and a point contact junction have opposite phases, which can be used to design a trijunction ring with π phase to probe the antiphase pairing. © 2009 The American Physical Society.published_or_final_versio

A movable image-based rendering system and its application to multiview audio-visual conferencing

Image-based rendering (IBR) is an emerging technology for rendering photo-realistic views of scenes from a collection of densely sampled images or videos. It provides a framework for developing revolutionary virtual reality and immersive viewing systems. This paper studies the design of a movable image-based rendering system based on a class of dynamic representations called plenoptic videos. It is constructed by mounting a linear array of 8 video cameras on an electrically controllable wheel chair with its motion being controllable manually or remotely through wireless LAN by means of additional hardware circuitry. We also developed a real-time object tracking algorithm and utilize the motion information computed to adjust continuously the azimuth or rotation angle of the movable IBR system in order to cope with a given moving object. Due to the motion of the wheel chair, videos may appear shaky and video stabilization technique is proposed to overcome this problem. The system can be used in a multiview audio-visual conferencing via a multiview TV display. Through this pilot study, we hope to develop a framework for designing movable IBR systems with improved viewing freedom and ability to cope with moving object in large environment. ©2010 IEEE.published_or_final_versionThe 10th International Symposium on Communications and Information Technologies (ISCIT 2010), Tokyo, Japan, 26-29 October 2010. In Proceedings of 10th ISCIT, 2010, p. 1142-114

Knowledge discovery for friction stir welding via data driven approaches: Part 2 – multiobjective modelling using fuzzy rule based systems

In this final part of this extensive study, a new systematic data-driven fuzzy modelling approach has been developed, taking into account both the modelling accuracy and its interpretability (transparency) as attributes. For the first time, a data-driven modelling framework has been proposed designed and implemented in order to model the intricate FSW behaviours relating to AA5083 aluminium alloy, consisting of the grain size, mechanical properties, as well as internal process properties. As a result, ‘Pareto-optimal’ predictive models have been successfully elicited which, through validations on real data for the aluminium alloy AA5083, have been shown to be accurate, transparent and generic despite the conservative number of data points used for model training and testing. Compared with analytically based methods, the proposed data-driven modelling approach provides a more effective way to construct prediction models for FSW when there is an apparent lack of fundamental process knowledge

Macroscopic Klein tunneling in spin-orbit-coupled Bose-Einstein condensates

We propose an experimental scheme to detect macroscopic Klein tunneling with spin-orbit-coupled Bose-Einstein condensates (BECs). We show that a nonlinear Dirac equation with tunable parameters can be realized with such BECs. Through numerical calculations, we demonstrate that macroscopic Klein tunneling can be clearly detected under realistic conditions. Macroscopic quantum coherence in such relativistic tunneling is clarified and a BEC with a negative energy is shown to be able to transmit transparently through a wide Gaussian potential barrier. © 2012 American Physical Society.published_or_final_versio

Current concepts in odontohypophosphatasia form of hypophosphatasia and report of two cases

published_or_final_versio

Cloning and characterization of a nitrite reductase gene related to somatic embryogenesis in Gossypium hirsutum

A nitrite reductase gene related to somatic embryogenesis was first cloned from Gossypium hirsutum. The cDNA sequence of the gene, named GhNiR, is 2,257 bp in length, with 254 bp of the 5&#8217; untranslated region and 236 bp of the 3&#8217; untranslated region. The open reading frame is 1,767 bp in length, encoding a deduced amino acid sequence of 588 residues with a molecular weight of 65.722 kDa and an isoelectric point of 7.07. Semi-quantitative RT-PCR analysis showed that the expression level of GhNiR was higher in embryogenic calli and somatic embryoids than in nonembryogenic calli among different somatic embryogenesis stages, and that the level of GhNiR mRNA was also higher in the cultivar with higher somatic embryogenesis ability. The catalytic GhNiR was verified by transformation in E. coli BL21 (DE3) strain with the recombinant expression vector pET-28A-GhNiR. NiR activity assay showedthat the crude GhNiR protein had obvious activity to NaNO2 substrate