On-site building of a Zn2＋-conductive interfacial layer via short-circuit energization for stable Zn anode

Aqueous zinc ion batteries(ZIBs)show great potential in large-scale energy storage systems for their advantages of high safety,low cost,high capacity,and enviro...     

等离子体的温度测量与光谱测温仪

论述了基于光谱学原理的温度测量技术．介绍了在上述原理下建立起来的光谱 测温仪。该仪器能测量符合局域热力学平衡与柱对称条件的电弧及高频等离子体的温度 剖面。测温范围为 ５ ０００—１５ ０００ Ｋ，不确定度约 ２．２％。     

加速度计应用

介绍了加速度计的基本工作原理及其在工业中的应用实例,可以为相关领域提供有用的技术参考。     

Application of EEG multipole model in the diagnosis of Alzheimer''''s disease

In order to find the electroencephalogram （EEG） characteristic parameters typical of Alzheimer＇s disease （AD） and explore an effective diagnostic method, a new mobile current multipole model was proposed to simulate the AD patient＇s cortical dipole source activities. The indicators of goodness of fit （GOF） and DtononD （DD） were calculated from EEG samples to evaluate the performance of this model. Relevant results showed that this multipole model with higher GOFvalues and larger DD change well fitted the pathological electrical activities of cortical neurons aroused by AD＇s extended sulcus and gyrus in the cerebral cortex. Meanwhile, the products of DD mean ＆ standard variance were found in a clear linear correlation with the diagnostic data of mini-mental state examination （MMSE） used in AD clinics. Furthermore, by tracing this multipole model＇s indicators in typical patients and contrasting with the functional magnetic resonance image （fMRI） as AD progressed, we suggested that the DD index may be suitable for monitoring the AD developments as a new diagnostic parameter.     

α－FeOOH超微粒的制备及热处理

利用化学沉淀法制备了α－ＦｅＯＯＨ超微粒，将其在不同温度下于空气氛中进行热处理，并使用ｘ－ｒａｙ衍射和透射电镜及热重分析仪对样品进行了测试与分析．     

Stepwise replication identifies a low-producing lymphotoxin-alpha allele as a major risk factor for early-onset leprosy

Host genetics has an important role in leprosy, and variants in the shared promoter region of PARK2 and PACRG were the first major susceptibility factors identified by positional cloning. Here we report the linkage disequilibrium mapping of the second linkage peak of our previous genome-wide scan, located close to the HLA complex. In both a Vietnamese familial sample and an Indian case-control sample, the low-producing lymphotoxin-alpha (LTA)+80 A allele was significantly associated with an increase in leprosy risk (P = 0.007 and P = 0.01, respectively). Analysis of an additional case-control sample from Brazil and an additional familial sample from Vietnam showed that the LTA+80 effect was much stronger in young individuals. In the combined sample of 298 Vietnamese familial trios, the odds ratio of leprosy for LTA+80 AA/AC versus CC subjects was 2.11 (P = 0.000024), which increased to 5.63 (P = 0.0000004) in the subsample of 121 trios of affected individuals diagnosed before 16 years of age. In addition to identifying LTA as a major gene associated with early-onset leprosy, our study highlights the critical role of case- and population-specific factors in the dissection of susceptibility variants in complex diseases.     

Germline gain-of-function mutations in SOS1 cause Noonan syndrome

Noonan syndrome, the most common single-gene cause of congenital heart disease, is characterized by short stature, characteristic facies, learning problems and leukemia predisposition. Gain-of-function mutations in PTPN11, encoding the tyrosine phosphatase SHP2, cause approximately 50% of Noonan syndrome cases. SHP2 is required for RAS-ERK MAP kinase (MAPK) cascade activation, and Noonan syndrome mutants enhance ERK activation ex vivo and in mice. KRAS mutations account for <5% of cases of Noonan syndrome, but the gene(s) responsible for the remainder are unknown. We identified missense mutations in SOS1, which encodes an essential RAS guanine nucleotide-exchange factor (RAS-GEF), in approximately 20% of cases of Noonan syndrome without PTPN11 mutation. The prevalence of specific cardiac defects differs in SOS1 mutation-associated Noonan syndrome. Noonan syndrome-associated SOS1 mutations are hypermorphs encoding products that enhance RAS and ERK activation. Our results identify SOS1 mutants as a major cause of Noonan syndrome, representing the first example of activating GEF mutations associated with human disease and providing new insights into RAS-GEF regulation.     

Recombination and linkage disequilibrium in Arabidopsis thaliana

Linkage disequilibrium (LD) is a major aspect of the organization of genetic variation in natural populations. Here we describe the genome-wide pattern of LD in a sample of 19 Arabidopsis thaliana accessions using 341,602 non-singleton SNPs. LD decays within 10 kb on average, considerably faster than previously estimated. Tag SNP selection algorithms and 'hide-the-SNP' simulations suggest that genome-wide association mapping will require only 40%-50% of the observed SNPs, a reduction similar to estimates in a sample of African Americans. An Affymetrix genotyping array containing 250,000 SNPs has been designed based on these results; we demonstrate that it should have more than adequate coverage for genome-wide association mapping. The extent of LD is highly variable, and we find clear evidence of recombination hotspots, which seem to occur preferentially in intergenic regions. LD also reflects the action of selection, and it is more extensive between nonsynonymous polymorphisms than between synonymous polymorphisms.     

Mutations in the gene encoding the basal body protein RPGRIP1L, a nephrocystin-4 interactor, cause Joubert syndrome

Protein-protein interaction analyses have uncovered a ciliary and basal body protein network that, when disrupted, can result in nephronophthisis (NPHP), Leber congenital amaurosis, Senior-L?ken syndrome (SLSN) or Joubert syndrome (JBTS). However, details of the molecular mechanisms underlying these disorders remain poorly understood. RPGRIP1-like protein (RPGRIP1L) is a homolog of RPGRIP1 (RPGR-interacting protein 1), a ciliary protein defective in Leber congenital amaurosis. We show that RPGRIP1L interacts with nephrocystin-4 and that mutations in the gene encoding nephrocystin-4 (NPHP4) that are known to cause SLSN disrupt this interaction. RPGRIP1L is ubiquitously expressed, and its protein product localizes to basal bodies. Therefore, we analyzed RPGRIP1L as a candidate gene for JBTS and identified loss-of-function mutations in three families with typical JBTS, including the characteristic mid-hindbrain malformation. This work identifies RPGRIP1L as a gene responsible for JBTS and establishes a central role for cilia and basal bodies in the pathophysiology of this disorder.