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1.
The germline carrier of the BRCA1 pathogenic mutation has been well proven to confer an increased risk of breast and ovarian cancer. Despite BRCA1 biallelic pathogenic mutations being extremely rare, they have been reported to be embryonically lethal or to cause Fanconi anemia (FA). Here we describe a patient who was a 48-year-old female identified with biallelic pathogenic mutations of the BRCA1 gene, with no or very subtle FA-features. She was diagnosed with ovarian cancer and breast cancer at the ages of 43 and 44 and had a strong family history of breast and gynecological cancers.  相似文献   
2.
Chemical durability of lanthanide zirconates (A2Zr2O7) (A = La-Yb) under near-field environments is important for evaluating their application as potential nuclear waste forms. In this work, A2Zr2O7 (A = La-Yb) are synthesized by spark plasma sintering with controlled microstructure and their chemical durability are evaluated in a nitric acid solution (pH = 1). Scanning transmission electron microscopy analysis reveals an amorphous passivation film either enriched with Zr or lanthanide. The complex chemistry of the passivation films can be correlated with a transition in corrosion mechanisms from a preferential release of lanthanide in La2Zr2O7 to a preferential release of Zr in Er2Zr2O7 and Yb2Zr2O7. These results suggest a dominant mechanism of incongruent dissolution and surface reorganization for the formation of passivation films. Strong correlations are identified between the leaching rates and cation ionic size, ionic potential, electronegativity differences between A-site cation and Zr, and bonding valence sum of oxygen, suggesting important impacts of structural and bonding characteristics in controlling chemical durability of lanthanide zirconates.  相似文献   
3.
Silicon - Quadruple gate FinFET is a promising candidate among other multi-gate MOS devices due to it’s better scalability and higher short channel effect suppression capability in advanced...  相似文献   
4.
Thyromimetics, whose physicochemical characteristics are analog to thyroid hormones (THs) and their derivatives, are promising candidates as novel therapeutics for neurodegenerative and metabolic pathologies. In particular, sobetirome (GC-1), one of the initial halogen-free thyromimetics, and newly synthesized IS25 and TG68, with optimized ADME-Tox profile, have recently attracted attention owing to their superior therapeutic benefits, selectivity, and enhanced permeability. Here, we further explored the functional capabilities of these thyromimetics to inhibit transthyretin (TTR) amyloidosis. TTR is a homotetrameric transporter protein for THs, yet it is also responsible for severe amyloid fibril formation, which is facilitated by tetramer dissociation into non-native monomers. By combining nuclear magnetic resonance (NMR) spectroscopy, computational simulation, and biochemical assays, we found that GC-1 and newly designed diphenyl-methane-based thyromimetics, namely IS25 and TG68, are TTR stabilizers and efficient suppressors of TTR aggregation. Based on these observations, we propose the novel potential of thyromimetics as a multi-functional therapeutic molecule for TTR-related pathologies, including neurodegenerative diseases.  相似文献   
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6.
Antimony triselenide (Sb2Se3) nanoflake-based nitrogen dioxide (NO2) sensors exhibit a progressive bifunctional gas-sensing performance, with a rapid alarm for hazardous highly concentrated gases, and an advanced memory-type function for low-concentration (<1 ppm) monitoring repeated under potentially fatal exposure. Rectangular and cuboid shaped Sb2Se3 nanoflakes, comprising van der Waals planes with large surface areas and covalent bond planes with small areas, can rapidly detect a wide range of NO2 gas concentrations from 0.1 to 100 ppm. These Sb2Se3 nanoflakes are found to be suitable for physisorption-based gas sensing owing to their anisotropic quasi-2D crystal structure with extremely enlarged van der Waals planes, where they are humidity-insensitive and consequently exhibit an extremely stable baseline current. The Sb2Se3 nanoflake sensor exhibits a room-temperature/low-voltage operation, which is noticeable owing to its low energy consumption and rapid response even under a NO2 gas flow of only 1 ppm. As a result, the Sb2Se3 nanoflake sensor is suitable for the development of a rapid alarm system. Furthermore, the persistent gas-sensing conductivity of the sensor with a slow decaying current can enable the development of a progressive memory-type sensor that retains the previous signal under irregular gas injection at low concentrations.  相似文献   
7.
猪肉是我国消耗量最大的肉类品,尤其是冷鲜猪肉更受人们青睐。猪胴体冷却保鲜加工工艺既要满足优质猪肉的成熟条件,又要保证猪肉的安全卫生。猪胴体冷却温度和降温速度对保证加工工艺合理性和降低耗能都至关重要。在实测数据基础上经过判断、分析提出,在常规0~4 ℃冷却前先在-10~-5 ℃低温快速冷却猪胴体1 h从而更好地保证猪胴体的冷却效果。实验发现:猪胴体开始冷却时,内部温度先有2~4 ℃的升温,然后才会降温,因此需对热负荷计算进行修正;根据两段冷却过程中猪胴体的冷却降温特点,提出了对应的冷量要求,为配备冷却装置提供了依据。  相似文献   
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9.
Sr-modified Cu/Nb co-doped BaTiO3 ceramics were prepared using solid-state reactions and the structures and dielectric properties were studied. All the samples had single-phase perovskite structures with no detectable secondary phases. In the low-temperature range, the dielectric constant decreased as the Sr content increased in the high- and low-frequency ranges. Two dielectric constant plateaus accompanied by dielectric relaxation peaks were present in the loss curves, and the relaxation process deviated from the Arrhenius law at low temperatures. The dielectric constants of different plateaus were related to inhomogeneous structures such as grain interiors and grain boundaries. The polarization strength of the grain boundaries in the low-frequency range increased with the temperature and that of the grain interiors demonstrated paraelectric behaviour in high-temperature ranges. An analysis of the electric modulus spectra indicated a close relationship between the relaxation process and resistivity of the grains for high-frequency relaxation. The impedance spectra at high temperatures consist of three electrical responses, corresponding to the effects of grains, grain boundaries, and electrodes. The dielectric relaxation appeared in high temperature range was related to the electrical properties of the grain boundaries.  相似文献   
10.
Inorganic nanoparticles (NPs) offer significant advantages to the biomedical field owing to their large surface area, controllable structures, diverse surface chemistry, and unique optical and physical properties. Researchers worldwide have shown that inorganic NPs and the released metal ions can act as therapeutic agents in targeted tissues or to cure various diseases without acute toxicity. In this progress report, the recent developments in inorganic NPs with different compositions directly used as therapeutics are discussed. First, the recent convergence of nanotechnology and biotechnology in biomedical applications as well as the unique functions, features, and advantages of inorganic NPs in biomedical applications are summarized. Thereafter, the biological effects of inorganic compositions in NPs which include balancing the intracellular redox environment, regulating the specific cellular signaling and cellular behaviors, and apoptosis are explained. In addition, the emerging therapeutic applications of inorganic NPs in various diseases are exemplified. Finally, the perspectives and challenges for overcoming the weaknesses of inorganic NPs as therapeutics are discussed. By carefully considering and investigating the biological effects of inorganic NPs and metal ions released from NPs, more promising inorganic NPs based therapeutic agents can be developed.  相似文献   
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