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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.
Two types of spherical zirconyl oxalate aqueous sols were successfully customized by a reverse micelles-mediated aqueous sol-gel process, and the sols were sequentially spin-coated on porous supports to prepare ZrO2 loose/tight bilayer ultrafiltration membranes. After three times of spin-coating process, a defect-free ZrO2 loose ultrafiltration membrane with pure water permeability of 110.5 ± 2.25 L m?2 h-1 bar-1, molecular weight cut-off (MWCO) of 16.5 kDa and excellent rejection of up to 97.5 % for bovine serum albumin was fabricated. Then, the loose ultrafiltration membrane was used as a substrate to prepare ZrO2 tight ultrafiltration membrane. Performances of tight ultrafiltration membrane regarding to permeability, retention of polyethylene glycol and treatment of dyes wastewater were evaluated. The tight ultrafiltration membrane with a thickness of 200 nm exhibited a pure water permeability of 22.5 ± 0.3 L m-2 h-1 bar-1 and MWCO of 1150 Da. Additionally, the rejections of methyl red and methyl orange by the tight ultrafiltration membrane were both <65 %, while of alizarin red, direct red, bromocresol green and methyl blue achieved maximum values of 98.5 %, 99.2 %, 99.5 % and 99.6 %, respectively. The fouled membranes could restore the virgin performance for reuse by cleaning and low-temperature calcination.  相似文献   
3.
Plant cell wall polysaccharides (PCWP) are abundantly present in the food of humans and feed of livestock. Mammalians by themselves cannot degrade PCWP but rather depend on microbes resident in the gut intestine for deconstruction. The dominant Bacteroidetes in the gut microbial community are such bacteria with PCWP-degrading ability. The polysaccharide utilization systems (PUL) responsible for PCWP degradation and utilization are a prominent feature of Bacteroidetes. In recent years, there have been tremendous efforts in elucidating how PULs assist Bacteroidetes to assimilate carbon and acquire energy from PCWP. Here, we will review the PUL-mediated plant cell wall polysaccharides utilization in the gut Bacteroidetes focusing on cellulose, xylan, mannan, and pectin utilization and discuss how the mechanisms can be exploited to modulate the gut microbiota.  相似文献   
4.
高熵形状记忆合金是在等原子比NiTi合金的基础上,结合高熵合金的概念,逐渐发展起来的一种新型高温形状记忆合金。近年来,已开发出了综合性能优异的(TiZrHf)50(NiCoCu)50系和(TiZrHf)50(NiCuPd)50系高熵形状记忆合金,引起了广泛的关注和研究兴趣。本文从物相组成、微观组织、马氏体相变行为、形状记忆效应和超弹性等角度出发,综述了高熵形状记忆合金的研究进展,并对高熵形状记忆合金未来的研究重点进行了展望。  相似文献   
5.
Increasing the dielectric loss capacity plays an important role in enhancing the electromagnetic absorption performance of materials. It remains a challenge to simultaneously introduce multiple types of dielectric losses in the material. In this work, we show that the atomic and interfacial dipole polarizations can be simultaneously enhanced by substituting N species into both carbon coating layers and bulk TiC lattices of a core-shell TiC@C material. Additionally, substitution of N species results more exposed TiC(111) facets and refines the TiC grain sizes in the bulk material, which is beneficial for enhancing the scattering of the external electromagnetic waves. The maximum reflection loss of the N substituted TiC@C material is measured as ?47.1 dB with an effective absorbing bandwidth of 4.83 GHz at 1.9 mm, which illustrates a valuable way to further tuning the electromagnetic absorption performance of this type of materials.  相似文献   
6.
阐述电磁安全的战略背景,分析电磁兼容到电磁安全的演化过程,剖析"自扰、互扰、敌扰"等现实迫切问题,阐明电磁干扰、电磁兼容等基本概念和相互关系,提出新需求、新技术、新趋势引发电磁兼容与电磁安全的"六大技术挑战",指出只有在认识和思维上系统性地实现"三个转变",才能实现电磁安全整体能力的提升,最后提出"电磁强国"的"五维布局"建议.  相似文献   
7.
Bone related diseases have caused serious threats to human health owing to their complexity and specificity. Fortunately, owing to the unique 3D network structure with high aqueous content and functional properties, emerging hydrogels are regarded as one of the most promising candidates for bone tissue engineering, such as repairing cartilage injury, skull defect, and arthritis. Herein, various design strategies and synthesis methods (e.g., 3D-printing technology and nanoparticle composite strategy) are introduced to prepare implanted hydrogel scaffolds with tunable mechanical strength, favorable biocompatibility, and excellent bioactivity for applying in bone regeneration. Injectable hydrogels based on biocompatible materials (e.g., collagen, hyaluronic acid, chitosan, polyethylene glycol, etc.) possess many advantages in minimally invasive surgery, including adjustable physicochemical properties, filling irregular shapes of defect sites, and on-demand release drugs or growth factors in response to different stimuli (e.g., pH, temperature, redox, enzyme, light, magnetic, etc.). In addition, drug delivery systems based on micro/nanogels are discussed, and its numerous promising designs used in the application of bone diseases (e.g., rheumatoid arthritis, osteoarthritis, cartilage defect) are also briefed in this review. Particularly, several key factors of hydrogel scaffolds (e.g., mechanical property, pore size, and release behavior of active factors) that can induce bone tissue regeneration are also summarized in this review. It is anticipated that advanced approaches and innovative ideas of bioactive hydrogels will be exploited in the clinical field and increase the life quality of patients with the bone injury.  相似文献   
8.
9.
4-methyl-2,4-bis(4-hydroxyphenyl)pent-1-ene (MBP), a major active metabolite of bisphenol A (BPA), is generated in the mammalian liver. Some studies have suggested that MBP exerts greater toxicity than BPA. However, the mechanism underlying MBP-induced pancreatic β-cell cytotoxicity remains largely unclear. This study demonstrated the cytotoxicity of MBP in pancreatic β-cells and elucidated the cellular mechanism involved in MBP-induced β-cell death. Our results showed that MBP exposure significantly reduced cell viability, caused insulin secretion dysfunction, and induced apoptotic events including increased caspase-3 activity and the expression of active forms of caspase-3/-7/-9 and PARP protein. In addition, MBP triggered endoplasmic reticulum (ER) stress, as indicated by the upregulation of GRP 78, CHOP, and cleaved caspase-12 proteins. Pretreatment with 4-phenylbutyric acid (4-PBA; a pharmacological inhibitor of ER stress) markedly reversed MBP-induced ER stress and apoptosis-related signals. Furthermore, exposure to MBP significantly induced the protein phosphorylation of JNK and AMP-activated protein kinase (AMPK)α. Pretreatment of β-cells with pharmacological inhibitors for JNK (SP600125) and AMPK (compound C), respectively, effectively abrogated the MBP-induced apoptosis-related signals. Both JNK and AMPK inhibitors also suppressed the MBP-induced activation of JNK and AMPKα and of each other. In conclusion, these findings suggest that MBP exposure exerts cytotoxicity on β-cells via the interdependent activation of JNK and AMPKα, which regulates the downstream apoptotic signaling pathway.  相似文献   
10.
Hydrodynamics characteristics of a fast and highly exothermic liquid–liquid oxidation process with in situ gas production in microreactors were studied using a newly developed experimental method. In the adipic acid synthesis through the K/A oil (the mixture of cyclohexanol and cyclohexanone) oxidation with nitric acid, bubble generation modes were divided into four categories. The gas production became more intensive, unstable, even explosive with increasing the oil phase feed rate and the temperature. A novel automatic image processing method was developed to monitor the instantaneous velocity online by tracking the gas–liquid interface. The axial velocity at the same location was unstable due to the changing gas production rate. Furthermore, the actual residence time was obtained easily with being only 36% of the space–time minimally, beneficial for establishing accurate kinetics and mass transfer models with time participation. Finally, an empirical correlation was developed to predict the actual residence time under different conditions.  相似文献   
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