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1. chinaXiv:201712.00929 [pdf]

Missense mutation R1345Q in CACNA1A gene causes a new type of ataxia with episodic tremor: clinical features, genetic analysis and treatment in a familial case

JIANG Haishan; WANG Dongmei; WANG Qun; YANG Man; WANG Wei; PAN Suyue; HU Yafang
Subjects: Medicine, Pharmacy >> Preclinical Medicine

Objective Mutations in CACNA1A, which encodes the P/Q-type calcium channel subunit, are responsible for at least 3 allelic diseases, namely type 2 episodic ataxia (EA-2), familial hemiplegic migraine type-1 (FHM1), and spinocerebellar ataxia type-6 (SCA 6). Herein we present a case of ataxia with episodic tremors in a 19-year-old man with a missense mutation of CACNA1A gene and summarize the clinical features, genetic analysis and treatment in this case and in his affected family members. Methods Physical examinations were conducted for the patient and his affected family members. DNA sample from the proband was analyzed with next-generation sequencing technology to identify the causative mutation. Sanger sequencing was used to confirm the gene mutation in the family members. Results Physical examinations of the patient revealed signs of ataxia, drunken gait, and tremor of his head and body. Four other members in his family had similar but much milder symptoms. A heterozygous missense mutation in CACNA1A (NM_001127221.1 c.4034G->A, p.R1345Q, exon 25) was identified in the proband, which was confirmed in the affected family members. The proband did not respond to methazolamide treatment, but his tremor symptom was well controlled with flunarizine, a calcium channel blocker. Conclusion Based on the clinical features, mutation analysis and treatment response, we suggest that this patient with a missense CACNA1A mutation, R1345Q, has a new type of ataxia with episodic tremor other than any of EA2, FHM1, or SCA 6.

submitted time 2017-12-27 From cooperative journals:《南方医科大学学报》 Hits388Downloads185 Comment 0

2. chinaXiv:201711.01914 [pdf]

Morphological coevolution of the pygostyle and tail feathers in Early Cretaceous birds

WANG Wei; Jingmai K. O’CONNOR
Subjects: Biology >> Zoology

The transformation from a long reptilian tail to a shortened tail ending in a pygostyle and accompanied by aerodynamic fanning rectrices is one of the most remarkable adaptations of early avian evolution. However, no fossils directly capture this transition, and information regarding the structural morphology and the early evolution of the pygostyle in Mesozoic birds and their integuments is relatively limited. Here we provide a review of the pygostyle morphology of Early Cretaceous birds with comparison to the structure in living birds. This study emphasizes the convergent evolution of distally co-ossified caudal vertebrae in non-avian maniraptorans and early birds. There further exist distinct differences in pygostyle morphology between Sapeornithiformes, Confuciusornithiformes, Enantiornithes, and Ornithuromorpha. The morphology of the pygostyle and rectrices in early ornithuromorphs appear similar to that of extant birds, whereas the pygostyle in more primitive birds does not appear morphologically capable of supporting the rectricial bulbs and musculature necessary to control an aerodynamic fan-shaped tail. The rectricial bulbs and rectricial fan appear to have coevolved with the plough-shaped pygostyle early in the evolution of the Ornithuromorpha. This study also shows that the confuciusornithiform pygostyle was more similar to that of enantiornithines than previously recognized, consistent with the presence of nearly identical ornamental tail feathers in both groups.

submitted time 2017-11-07 From cooperative journals:《古脊椎动物学报》 Hits313Downloads170 Comment 0

3. chinaXiv:201711.00130 [pdf]

Investigation of the Substituent Effects on π-Type Pnicogen Bond Interaction

XU Hui-Ying; CAO Sheng-Wei; WANG Wei; ZHU Jian-Qing; ZOU Jian-Wei; XU Xiao-Lu LU Yin
Subjects: Chemistry >> Physical Chemistry

Intermolecular interactions between PH2Cl and Ar–R (R = H, OH, NH2, CH3, Br, Cl, F, CN, NO2) were calculated by using MP2/aug-cc-pVDZ quantum chemical method. It has been shown from our calculations that the aromatic rings with electron-withdrawing groups represent much weaker binding affinities than those with electron-donating groups. The charge-transfer interaction between PH2Cl and Ar–R plays an important role in the formation of pnicogen bond complexes, as revealed by NBO analysis. Nevertheless, AIM analysis shows that the nature of the interactions between PH2Cl and Ar–R is electrostatic, and the interaction energies of the complexes are correlated positively with the electron densities in the bond critical points (BCPs). RDG/ELF graphical analyses were performed to visualize the positions and strengths of the pnicogen bonding, as well as the spatial change of the electron localization upon the formation of complexes. The π-type halogen bond was also calculated, and it has been revealed that the π-type pnicogen bond systems are more stable than the halogen bond ones.

submitted time 2017-11-05 From cooperative journals:《结构化学》 Hits1286Downloads632 Comment 0

4. chinaXiv:201711.01119 [pdf]

The Study Featuresof Variations of Cyclotron in GX 301一2 by using the Resonant Scattering INTEGRAL/IBIS

Yu Guangwen; Wang Wei
Subjects: Astronomy >> Astrophysical processes

With long-term hard X-ray monitoring,observations on high mass X-ray binary GX 301-2 from 2003一2011 performed by INTEGRAL/IBIS,we first systematically studied its spectral properties in different accreting luminosities and orbital phases. The cyclotron resonant scattering feature(CRSF) at energies from 35一47 keV was detected in the hard X-ray spectra,suggesting the magnetic field of Sx10}Z Gs. The variations of the CRSF show no relation to the X-ray luminosity while the line centroid energy of the CRSF has a positive correlation to the photon index and spectral cutoff energy,and there also exists a weak correlation between the absorption depth and cutoff energy. These relations support the idea that the spectral cutoff in accreting X-ray pulsars is strongly affected by the cyclotron resonant scattering. The correlation between the ratio of the line width to the centroid energy and absorption depth implies a tall cylindrical accreting column on the surface of the neutron star in GX 301-2. The explanation of the long spin period in GX 301-2 requires a strong surface magnetic field at least higher than 2x 1014 Gs which is contradict with the measured value based on the cyclotron line energy. The line-forming region with a height at least larger than two neutron star radii is proposed to resolve the contradiction. This scenario is supported by the tall cylindrical accreting column on the neutron star surface in GX 301-2 according to the variation patterns of the CRSF. In addition,the possible evolution scenario of accreting magnetars like GX 301-2 is briefly discussed,and GX 301-2 would he an accreting magnetar in the equivalence phase.

submitted time 2017-10-20 From cooperative journals:《天文研究与技术》 Hits4349Downloads399 Comment 0

5. chinaXiv:201708.00336 [pdf]

Morphological coevolution of the pygostyle and tail feathers in Early Cretaceous birds

WANG Wei; Jingmai K. O'CONNOR
Subjects: Geosciences >> Geology

The transformation from a long reptilian tail to a shortened tail ending in a pygostyle and accompanied by aerodynamic fanning rectrices is one of the most remarkable adaptations of early avian evolution. However, no fossils directly capture this transition, and information regarding the structural morphology and the early evolution of the pygostyle in Mesozoic birds and their integuments is relatively limited. Here we provide a review of the pygostyle morphology of Early Cretaceous birds with comparison to the structure in living birds. This study emphasizes the convergent evolution of distally co-ossified caudal vertebrae in non-avian maniraptorans and early birds. There further exist distinct differences in pygostyle morphology between Sapeornithiformes, Confuciusornithiformes, Enantiornithes, and Ornithuromorpha. The morphology of the pygostyle and rectrices in early ornithuromorphs appear similar to that of extant birds, whereas the pygostyle in more primitive birds does not appear morphologically capable of supporting the rectricial bulbs and musculature necessary to control an aerodynamic fan-shaped tail. The rectricial bulbs and rectricial fan appear to have coevolved with the plough-shaped pygostyle early in the evolution of the Ornithuromorpha. This study also shows that the confuciusornithiform pygostyle was more similar to that of enantiornithines than previously recognized, consistent with the presence of nearly identical ornamental tail feathers in both groups. 从兽脚类恐龙中爬行类的骨质长尾,到以尾综骨为终端,并附着具有空气动力学功能的扇状尾羽的短巧尾部,是早期鸟类演化中最显著的适应性转变之一。但能直接反映这一转变的化石记录匮乏,而且对中生代鸟类尾部形态结构,以及尾综骨和尾羽早期演化的认知也相对不足。在此对早白垩世鸟类的尾综骨形态予以概述并将其与现生鸟类尾部结构类比。本研究强调了非鸟手盗龙类中尾椎的联合骨化与早期鸟类的尾综骨实属趋同演化。本研究表明,会鸟形类、孔子鸟形类、反鸟类和今鸟型类的尾综骨结构存在明显差异。今鸟型类尾综骨和尾羽(舵羽)与现代鸟类的相似,而相对更原始的鸟类的尾综骨,从形态来看,并不能支持舵羽球状膨大和必要的肌肉附着来操控具有空气动力学功能的扇状尾羽。由此可见,舵羽球状膨大、舵羽扇面与犁铧状的尾综骨是在今鸟型类演化早期相伴相生的。相对于从前的认知,本研究还发现孔子鸟类的尾综骨与反鸟类的有更多相似之处,与二者都具有的几乎相同的装饰性尾羽相符合。

submitted time 2017-08-24 From cooperative journals:《古脊椎动物学报》 Hits2520Downloads582 Comment 0

6. chinaXiv:201705.00588 [pdf]

Porosity effect on ZrO2 hollow shells and hydrothermal stability for catalytic steam reforming of methane

Lim, ZY [Lim, Zi-Yian][ 2,1 ]; Wu, CZ [Wu, Chunzheng][ 1 ]; Wang, WG [Wang, Wei Guo][ 1 ]; Choy, KL [Choy, Kwang-Leong][ 3 ]; Yin, HF [Yin, Hongfeng][ 1 ]
Subjects: Physics >> General Physics: Statistical and Quantum Mechanics, Quantum Information, etc.

Hydrogen is an emerging energy source/carrier for oil refining and fuel cell applications. The development of an efficient and stable catalyst to produce hydrogen-rich gas is required for industrial application. The Ni@yolk-ZrO2 catalyst could be a potential solution to tackle the challenges in hydrogen production. The catalyst was characterized using a combination of XRD, TEM, AAS, TPR, BET, and XPS. In this study, the amount of micropores in ZrO2 hollow shells was demonstrated to influence the catalytic performance. Ni@yolk-ZrO2 catalysts were evaluated for 48 hours under steam reforming of methane and their porosity effect in ZrO2 hollow shells was identified. From the characterization of BET and catalytic evaluation, the physical information of the ZrO2 hollow shell was established, which affected the catalytic performance in steam reforming of methane. Furthermore, the results from XPS and TEM showed that Ni particles were controlled under a ZrO2 yolk–shell structure framework and showed the characteristic of moderately strong hydrothermal stability after the steam reforming test. The catalysts were studied at a GHSV of 50 400 mL gcat 1 h 1 and S/C 1⁄4 2.5 at 750 C and they remained stable with methane conversion more than 90% for 48 hours.

submitted time 2017-05-02 Hits910Downloads232 Comment 0

7. chinaXiv:201705.00584 [pdf]

Synthesis and characterization of transparent polyimides derived from ester-containing dianhydrides with different electron affinities

Zhou, Y [Zhou, Yu][ 1,2 ]; Chen, GF [Chen, Guofei][ 1 ]; Wang, W [Wang, Wei][ 1 ]; Wei, LH [Wei, Lihong][ 1 ]; Zhang, QJ [Zhang, Qiuju][ 1 ]; Song, LP [Song, Liping][ 2 ]; Fang, XZ [Fang, Xingzhong][ 1 ]
Subjects: Physics >> General Physics: Statistical and Quantum Mechanics, Quantum Information, etc.

Two series of poly(ester imide)s derived from bis(trimellitic acid anhydride) phenyl ester (TAHQ) and bis[(3,4- dicarboxylic anhydride) phenyl] terephthalate (PAHP), as well as poly(ether imide)s based on hydroquinone diphthalic anhydride (HQDPA), were synthesized with aromatic diamines via solution polycondensation. These polyimide films were transparent with an ultraviolet-visible absorption cut-off wavelength below 375 nm, and with tensile strengths of 42.0–83.8 MPa, tensile moduli of 2.5–4.7 GPa and elongations at break of 2.1–5.4%. Compared with the poly(ether imide)s, the poly(ester imide)s showed higher glass transition temperatures (Tg), lower water absorption (WA) and lower temperature of 5% weight loss (Td5%). Moreover, the poly(ester imide)s derived from PAHP with a low electron affinity of 2.04 eV by theoretical calculation achieved better transparency, lower WA and slightly lower Tg than the corresponding TAHQ- based poly(ester imide)s.

submitted time 2017-05-02 Hits566Downloads284 Comment 0

8. chinaXiv:201705.00520 [pdf]

Green synthesis of nanoporous Si/C anode using NaCl template with improved cycle life

Fan, X [Fan, Xu][ 1,2 ]; Jiang, XP [Jiang, Xiangping][ 1 ]; Wang, W [Wang, Wei][ 2 ]; Liu, ZP [Liu, Zhaoping][ 2 ]
Subjects: Physics >> General Physics: Statistical and Quantum Mechanics, Quantum Information, etc.

Porous silicon/carbon (Si/C) structure has been proved of great help to enhance the electrochemical cycling performance of Si-based anode materials. Current fabrication of porous Si based anodes usually relies on the complicated chemical etching process with unavoidable pollution problem. Herein, a na- noporous Si/C composite was successfully fabricated by a facile spray drying and carbonization process. The environmentally benign NaCl is used as the sacrificing template for the generation of embedded nanopores, which can be easily removed by routine washing process. The as-prepared nanoporous Si/C anode exhibits a high reversible specific capacity of around 900 mAh g 1 with 78.2% capacity retention after 100 cycles. This synthetic method paves a simple and environment-friendly way to build porous Si based anode with improved electrochemical performance which is significantly important for the in- dustrial production.

submitted time 2017-05-02 Hits435Downloads235 Comment 0

9. chinaXiv:201705.00437 [pdf]

Quantitative contribution of resistance sources of components to stack performance for solid oxide electrolysis cells

Zheng, YF [Zheng, Yifeng][ 1 ]; Li, QS [Li, Qingshan][ 1 ]; Chen, T [Chen, Tao][ 1 ]; Xu, C [Xu, Cheng][ 1 ]; Wang, WG [Wang, Wei Guo][ 1 ]
Subjects: Physics >> General Physics: Statistical and Quantum Mechanics, Quantum Information, etc.

Quantitative investigation is conducted on the resistance sources of the components in the NiOeYSZ/ YSZ/GDC/LSCFeGDC solid oxide electrolysis cell (SOEC) stack at the H2O/H2 ratios of 70/30, 80/20 and 90/ 10 at 750 C. The results indicate that the cell resistance accounts for 76.3e66.7% of that of the stack repeating unit (SRU), the contact resistance (CR) between the air electrode current-collecting layer (AECCL) and the interconnect accounts for 23.6e27.0%, the CR between the hydrogen electrode current- collecting layer (HECCL) and the interconnect only accounts for 2.3e3.2%, and the resistance of the interconnect can be neglected. Duration test of the stack is conducted at 0.8 A cm 2 for 380 h, the cell resistance increase is found to be the major contribution of the SRU degradation (82.2% of the SRU degradation) while the air electrode CR increase and the hydrogen electrode CR increase are other two important factors.

submitted time 2017-05-02 Hits277Downloads160 Comment 0

10. chinaXiv:201705.00427 [pdf]

Synthesis, Characterization, and Catalytic Activity of Mn-doped Perovskite Oxides for Three-Way Catalysis

Zhou, C [Zhou, Chen][ 1 ]; Zhang, YX [Zhang, Yexin][ 1 ]; Hu, LJ [Hu, Lingjun][ 1 ]; Yin, HF [Yin, Hongfeng][ 1 ]; Wang, WG [Wang, Wei Guo][ 1 ]

Mn-doped La0.8Sr0.2CoO3 perovskite oxides [La0.8Sr0.2Co1-xMnxO3; x = 0, 0.1, 0.3, 0.5] were synthesized by a modified sol-gel method. The phase-pure oxides were obtained. CoO and carbonates were formed on the surface of La0.8Sr0.2CoO3. With increasing dop

submitted time 2017-05-02 Hits171Downloads114 Comment 0

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