Spinocerebellar ataxias （SCAs） are a group of genetic disorders characterized by slowly progressive incoordina- tion of gait and are often associated with poor coordination of the hands, speech, and eye movements. F...Spinocerebellar ataxias （SCAs） are a group of genetic disorders characterized by slowly progressive incoordina- tion of gait and are often associated with poor coordination of the hands, speech, and eye movements. Frequently, atrophy of the cerebellum occurs. The genetic forms of ataxia are diagnosed by family history, physical examina- tion, neuroimaging, and molecular genetic testing. At present, 36 SCA subtypes including 27 pathogenic genes have been identified [1]. Different subtypes of SCAs have clear distribution differences among ethnic populations, and SCA8 is an infrequent entity worldwide, which has mostly been reported in Japanese, but has never been reported in Chinese [2]. SCAB involves bidirectional expression based on the total number of both the （CTA）n and （CTG）n expansion transcripts in ATXN8OS. The pathogenesis of this disorder is complex and the spectrum of clinical presentations is broad. It is predominantly characterized by drawn-out slowness of speech and gait instability, followed by slowly progressive ataxia, with disease onset typically occurring in adulthood [3]. How- ever, the lowest full-penetrance allele for SCA8 onset remains elusive and the current understanding of the phenotypic and genotypic features of SCA8 is limited. Since SCA8 has not yet been reported in the Chinese population and is scantily reported in a small proportion of pedigrees so far, clinical knowledge is still developing. Moreover, the boundary between the normal and patho- genic alleles of SCA8 is uncertain. Here we report the clinical and molecular genetic characteristics of 3 Chinese SCA8 families and have identified 51 CTA/CTG repeats within ATXN8OS, probably the shortest pathogenic allele for SCA8.展开更多
The noise data in vertical component records of 85 seismic stations in Fujian Province during 2012 is used as the research object in this paper. The noise data is divided into fiveminute segments to calculate the powe...The noise data in vertical component records of 85 seismic stations in Fujian Province during 2012 is used as the research object in this paper. The noise data is divided into fiveminute segments to calculate the power spectra. The high reference line and low reference line of station are then identified by drawing a probability density function graph( PDF)using the power spectral probability density function. Moreover, according to the anomalies of PDF graphs in 85 seismic stations,the abnormal noise is divided into four categories: dropped packet, low noise, high noise, and median noise anomalies.Afterwards,four selection methods are found by the high or low noise reference line of the stations,and the system of real-time monitoring of seismic noise is formed by combining the four selection methods. Noise records of 85 seismic stations in Fujian Province in July2013 are selected for verification,and the results show that the anomalous noise-recognition system could reach a 90% success rate at most stations and the effect of selection are very good. Therefore,it could be applied to the seismic noise real-time monitoring in stations.展开更多
Based on the analysis and research of the airworthiness objective of integrated modular avionics system(IMA),and the characteristics of IMA system’s comprehensive and complex cross-linking with other airborne systems...Based on the analysis and research of the airworthiness objective of integrated modular avionics system(IMA),and the characteristics of IMA system’s comprehensive and complex cross-linking with other airborne systems,the extraction strategy of IMA system’s compliance flight test subjects and the selection method of IMA system’s compliance flight test parameters are proposed.The data analysis method based on the abnormal probability matrix of the IMA system is proposed for the first time,and the abnormal state information of the IMA system can be quickly identified.The compliance flight test of the IMA system is completed with limited flight test resources,which achieves the purpose of saving flight test sorties and improving flight test efficiency.This research has been successfully applied to the airworthiness certification flight test of a certain civil transport aircraft in China,and can provide technical support for the subsequent type flight test.展开更多
Dear Editor,As an important energy storage device,lithium-ion battery plays a vital role in electric aircrafts,which are new and promising equipment of transportation in the future with low carbon emissions.Accurate p...Dear Editor,As an important energy storage device,lithium-ion battery plays a vital role in electric aircrafts,which are new and promising equipment of transportation in the future with low carbon emissions.Accurate prediction of the state of charge(SOC)of lithium-ion batteries is of great importance in reducing the probability of abnormal accidents and ensuring flight safety.展开更多
基金supported by grants from the National Natural Science Foundation of China(81301486 and81672095)
文摘Spinocerebellar ataxias （SCAs） are a group of genetic disorders characterized by slowly progressive incoordina- tion of gait and are often associated with poor coordination of the hands, speech, and eye movements. Frequently, atrophy of the cerebellum occurs. The genetic forms of ataxia are diagnosed by family history, physical examina- tion, neuroimaging, and molecular genetic testing. At present, 36 SCA subtypes including 27 pathogenic genes have been identified [1]. Different subtypes of SCAs have clear distribution differences among ethnic populations, and SCA8 is an infrequent entity worldwide, which has mostly been reported in Japanese, but has never been reported in Chinese [2]. SCAB involves bidirectional expression based on the total number of both the （CTA）n and （CTG）n expansion transcripts in ATXN8OS. The pathogenesis of this disorder is complex and the spectrum of clinical presentations is broad. It is predominantly characterized by drawn-out slowness of speech and gait instability, followed by slowly progressive ataxia, with disease onset typically occurring in adulthood [3]. How- ever, the lowest full-penetrance allele for SCA8 onset remains elusive and the current understanding of the phenotypic and genotypic features of SCA8 is limited. Since SCA8 has not yet been reported in the Chinese population and is scantily reported in a small proportion of pedigrees so far, clinical knowledge is still developing. Moreover, the boundary between the normal and patho- genic alleles of SCA8 is uncertain. Here we report the clinical and molecular genetic characteristics of 3 Chinese SCA8 families and have identified 51 CTA/CTG repeats within ATXN8OS, probably the shortest pathogenic allele for SCA8.
基金sponsored by the National Key Technology R&D Program of China(2009BAK55B00)the Earthquake Industry Research Project(201508012)
文摘The noise data in vertical component records of 85 seismic stations in Fujian Province during 2012 is used as the research object in this paper. The noise data is divided into fiveminute segments to calculate the power spectra. The high reference line and low reference line of station are then identified by drawing a probability density function graph( PDF)using the power spectral probability density function. Moreover, according to the anomalies of PDF graphs in 85 seismic stations,the abnormal noise is divided into four categories: dropped packet, low noise, high noise, and median noise anomalies.Afterwards,four selection methods are found by the high or low noise reference line of the stations,and the system of real-time monitoring of seismic noise is formed by combining the four selection methods. Noise records of 85 seismic stations in Fujian Province in July2013 are selected for verification,and the results show that the anomalous noise-recognition system could reach a 90% success rate at most stations and the effect of selection are very good. Therefore,it could be applied to the seismic noise real-time monitoring in stations.
文摘Based on the analysis and research of the airworthiness objective of integrated modular avionics system(IMA),and the characteristics of IMA system’s comprehensive and complex cross-linking with other airborne systems,the extraction strategy of IMA system’s compliance flight test subjects and the selection method of IMA system’s compliance flight test parameters are proposed.The data analysis method based on the abnormal probability matrix of the IMA system is proposed for the first time,and the abnormal state information of the IMA system can be quickly identified.The compliance flight test of the IMA system is completed with limited flight test resources,which achieves the purpose of saving flight test sorties and improving flight test efficiency.This research has been successfully applied to the airworthiness certification flight test of a certain civil transport aircraft in China,and can provide technical support for the subsequent type flight test.
基金supported in part by the Chunhui Project of the Ministry of Education of China(HZKY20220429)the Department of Science&Technology of Liaoning Province(2022-MS-300)the Educational Department of Liaoning Province(LJKMZ20220561)
文摘Dear Editor,As an important energy storage device,lithium-ion battery plays a vital role in electric aircrafts,which are new and promising equipment of transportation in the future with low carbon emissions.Accurate prediction of the state of charge(SOC)of lithium-ion batteries is of great importance in reducing the probability of abnormal accidents and ensuring flight safety.
