Novel ACTG1 mutation causing autosomal dominant non-syndromic hearing impairment in a Chinese family

γ -actin （ACTG1） gene is a cytoplasmic nonmuscle actin gene, which encodes a major cytoskeletal protein in the sensory hair cells of the cochlea. Mutations in ACTG1 were found to cause autosomal dominant, progressive, sensorineural hearing loss linked to the DFNA 20/26 locus on chromosome 17q25.3 in European and American families, respectively. In this study, a novel missense mutation （c.364A〉G; p.I122V） co-segregated with the affected individuals in the family and did not exist in the unaffected family members and 150 unrelated normal controls. The alteration of residue Ile122 was predicted to damage its interaction with actin-binding proteins, which may cause disruption of hair cell organization and function. These findings strongly suggested that the I122V mutation in ACTG1 caused autosomal dominant non-syndromic hearing impairment in a Chinese family and expanded the spectrum of ACTG1 mutations causing hearing loss.

Diffusion tensor imaging study of fractional anisotropy values and the area of both cerebral peduncles in healthy adults

Diffusion tensor imaging was performed in 105 volunteers free of central nervous system lesions.No differences were found in fractional anisotropy between the left and right cerebral peduncles among subjects（P 0.05）.The lower limit value of fractional anisotropy was 0.36,and the asymmetry ratio was 0.77.The area and lower limit value of the cerebral peduncles were 0.90 cm2 and 0.83,respectively.These results will be useful in evaluating the diagnosis of Wallerian degeneration following cerebral infarction.

Why do stroke patients with negative motor evoked potential show poor limb motor function recovery?

Negative motor evoked potentials after cerebral infarction, indicative of poor recovery of limb motor function, tend to be accompanied by changes in fractional anisotropy values and the cerebral pe-duncle area on the affected side, but the characteristics of these changes have not been reported. This study included 57 cases of cerebral infarction whose motor evoked potentials were tested in the 24 hours after the first inspection for diffusion tensor imaging, in which 29 cases were in the negative group and 28 cases in the positive group. Twenty-nine patients with negative motor evoked potentials were divided into two groups according to fractional anisotropy on the affected side of the cerebral peduncle： a fractional anisotropy 〈 0.36 group and a fractional anisotropy 〉 0.36 group. All patients underwent a regular magnetic resonance imaging and a diffusion tensor imaging examina- tion at 1 week, 1, 3, 6 and 12 months after cerebral infarction. The FugI-Meyer scores of their hemiplegic limbs were tested before the magnetic resonance and diffusion tensor imaging exami-nations. In the negative motor evoked potential group, fractional anisotropy in the affected cerebral peduncle declined progressively, which was most obvious in the first 1-3 months after the onset of cerebral infarction. The areas and area asymmetries of the cerebral peduncle on the affected side were significantly decreased at 6 and 12 months after onset. At 12 months after onset, the area asymmetries of the cerebral peduncle on the affected side were lower than the normal lower limit value of 0.83. FugI-Meyer scores in the fractional anisotropy ≥0.36 group were significantly higher than in the fractional anisotropy 〈 0.36 group at 3-12 months after onset. The fractional anisotropy of the cerebral peduncle in the positive motor evoked potential group decreased in the first 1 month after onset, and stayed unchanged from 3-12 months; there was no change in the area of the cerebral peduncle in the first 1-12 months after cerebral infarction. These findings confirmed that if the fractional anisotropy of the cerebral peduncle on the affected side is 〈 0.36 and the area asym-metries 〈 0.83 in patients with negative motor evoked potential after cerebral infarction, then poor hemiplegic limb motor function recovery may occur.

Design strategies and recent advancements of solid-state supercapacitor operating in wide temperature range

Solid-state supercapacitors(SSCs)are emerging as one of the promising energy storage devices due to their high safety,superior power density,and excellent cycling life.However,performance degradation and safety issues under extreme conditions are the main challenges for the practical application.With the expansion of human activities,such as space missions,polar exploration,and so on,the investigation of SSC with wide temperature tolerance,high energy density,power density,and sustainability is highly desired.In this review,the effects of temperature on SSC are systematically illustrated and clarified,including the properties of the electrolyte,ion diffusion,and reaction dynamics of the supercapacitor.Subsequently,we summarize the recent advances in wide-temperature-range SSCs from the aspect of electrolyte modification,electrode design,and interface adjustment between electrode and electrolyte,especially with critical concerns on ionic conductivity and cycling stability.In the end,a perspective is presented,expecting to promote the practical application of the SSC in harsh conditions.

低维结构策略构筑基于钙钛矿纳米片/氧化锌纳米线的柔性宽光谱探测器（英文）

柔性光电探测器具有轻便、易携带和优异的大面积兼容性等特点,在下一代光电子器件领域具有巨大的应用潜力.柔性光电探测器面临的主要挑战是在反复弯曲、拉伸、折叠等形变状态下难以保持优异的性能.本文通过低维度结构策略构筑了基于CsPbBr3纳米片和ZnO纳米线的柔性光电探测器.得益于一维纳米线和二维纳米片的高柔性,所构筑的光电探测器在各种应力下表现出优异的工作稳定性.例如,在弯曲1000次之后,器件的性能没有明显变化.此外,由于ZnO和CsPbBr3自身的光吸收特性,所构筑的柔性光电探测器展现出宽光谱光电响应能力(涵盖紫外和可见波段).在紫外和可见区域的峰值响应度分别为3.10和0.97 A W^-1,其相应的探测率分别为5.57×10^12和1.71×10^12Jones.本文针对柔性、高性能集成光电探测器提出的维度构筑策略,在未来智能、可穿戴光电子器件领域有着巨大的应用前景.

Fiber Solar Cells from High Performances Towards Real Applications

The evolution of electronic systems towards small,flexible,portable and human-centered forms drives the demand for onbody power supplies with lightweight and high flexibility.Fiber solar cells that can be integrated into soft and lightweight textiles are considered as potential sustainable power sources for the next generation of wearable electronics.To this end,they have been extensively investigated in the past decade aiming to improve their photovoltaic performances,but there is still a big gap between the high-performance devices and real applications.Herein,the key advances of configurations,fabrications and performances of fiber solar cells are highlighted and analyzed.Based on the current progress,the latest ideas with regard to the remaining challenges and opportunities beyond the reach of the previous studies are presented.

人类衰竭心脏中髓系细胞的全面描述

Heart failure(HF)remains a rising global epidemic worldwide[1,2].It is widely recognized that immune cells play important roles in the pathogenesis of HF.The hearts of HF patients are characterized by persistent low-grade inflammation,which has been linked to adverse ventricular remodeling as it promotes interstitial fibrosis and cardiomyocyte dysfunction and loss.