Patient with unusual Hutchinson-Gilford syndrome (progeria)

A patient with unusual Hutchinson-Gilford syndrome (progeria) is reported. This 7-year-old boy had all the characteristics of progeria, except for coxa valga and the "horse-riding" stance. A previous cerebral infarction was detected in the right putamen on cranial magnetic resonance imaging. During treadmill exercise test electrocardiography, ST depression suggested the existence of arteriosclerotic lesions. Skin fibroblast culture exhibited 76% DNA-repair capacity compared to normal. He has not manifested endocrinologic abnormalities. From these findings it is concluded that this patient has an incomplete case of Hutchinson-Gilford syndrome and that a correlation may exist between the clinical features and the degree of DNA-repair capacity.     

高质量密度储氢材料-氨基化合物的合成

采用真空蒸发法制备了氨基化锂LiNH2(+2·LiH)薄膜,即首先将Li真空镀膜到金属基板上,然后经氮化、氢化处理合成LiNH2薄膜.此外,真空蒸发法也用于Mg部分置换Li后薄膜的制备.实验结果证明,与传统的粉末状试样相比,薄膜状试样能够在更低的温度下释放出氢气.研究表明采用包括真空蒸发法等非传统方法合成金属络合物储氢材料对进一步提高其储放氢特性具有良好的效果.     

A new control strategy for optimum-efficiency operation of asynchronous reluctance motor

In this paper, an optimum-efficiency control scheme of synchronous reluctance motors is presented. There exists a variety of combinations of d- and q-axis current which provides a specific motor torque. The objective of the optimum-efficiency controller is to seek a combination of d- and q-axis current components, which provides minimum input power, that is, minimum losses at a certain operating point in steady state. A small amount of perturbation is added to the d-axis current reference for the purpose of searching a minimum input power operating point     

Back‐channel‐etched thin‐film transistor using c‐axis‐aligned crystal In–Ga–Zn oxide

Our crystalline In–Ga–Zn oxide (IGZO) thin film has a c‐axis‐aligned crystal (CAAC) structure and maintains crystallinity even on an amorphous base layer. Although the crystal has c‐axis alignment, its a‐axis and b‐axis have random arrangement; moreover, a clear grain boundary is not observed. We fabricated a back‐channel‐etched thin‐film transistor (TFT) using the CAAC‐IGZO film. Using the CAAC‐IGZO film, more stable TFT characteristics, even with a short channel length, can be obtained, and the instability of the back channel, which is one of the biggest problems of IGZO TFTs, is solved. As a result, we improved the process of manufacturing back‐channel‐etched TFTs.     

Genome Editing Using Cas9 Ribonucleoprotein Is Effective for Introducing PDGFRA Variant in Cultured Human Glioblastoma Cell Lines

Many variants of uncertain significance (VUS) have been detected in clinical cancer cases using next-generation sequencing-based cancer gene panel analysis. One strategy for the elucidation of VUS is the functional analysis of cultured cancer cell lines that harbor targeted gene variants using genome editing. Genome editing is a powerful tool for creating desired gene alterations in cultured cancer cell lines. However, the efficiency of genome editing varies substantially among cell lines of interest. We performed comparative studies to determine the optimal editing conditions for the introduction of platelet-derived growth factor receptor alpha (PDGFRA) variants in human glioblastoma multiforme (GBM) cell lines. After monitoring the copy numbers of PDGFRA and the expression level of the PDGFRα protein, four GBM cell lines (U-251 MG, KNS-42, SF126, and YKG-1 cells) were selected for the study. To compare the editing efficiency in these GBM cell lines, the modes of clustered regularly interspaced short palindromic repeat (CRISPR)-associated protein 9 (Cas9) delivery (plasmid vs. ribonucleoprotein (RNP)), methods of transfection (lipofection vs. electroporation), and usefulness of cell sorting were then evaluated. Herein, we demonstrated that electroporation-mediated transfer of Cas9 with single-guide RNA (Cas9 RNP complex) could sufficiently edit a target nucleotide substitution, irrespective of cell sorting. As the Cas9 RNP complex method showed a higher editing efficiency than the Cas9 plasmid lipofection method, it was the optimal method for single-nucleotide editing in human GBM cell lines under our experimental conditions.     

A study of the critical nozzle for flow rate measurement of high-pressure hydrogen gas

The mass flow rate measurement using a critical nozzle shows the validity of the inviscid theory,indicating thatthe discharge coefficient increases and approaches unity as the Reynolds number increases under the ideal gas lawHowever,when the critical nozzle measures the mass flow rate of a real gas such as hydrogen at a pressure ofhundreds bar,the discharge coefficient exceeds unity,and the real gas effects should be taken into account.Thepresent study aims at investigating the flow features of the critical nozzle using high-pressured hydrogen gas.Theaxisymmetric,compressible Navier-Stokes computation is employed to simulate the critical nozzle flow,and afully implicit finite volume method is used to discretize the governing equation system.The real gas effects aresimulated to consider the intermolecular forces,which account for the possibility of liquefying hydrogen gas.Thecomputational results are compared with past experimental data.It has been found that the coefficient of dis-charge for real gas can be corrected properly below unity adopting the real gas assumption.