Arthrogryposis, renal dysfunction and cholestasis (ARC) syndrome is a rare genetic disorder and has not been described in China. We present a female infant with neonatal intrahepatic cholestasis from a Chinese family ...Arthrogryposis, renal dysfunction and cholestasis (ARC) syndrome is a rare genetic disorder and has not been described in China. We present a female infant with neonatal intrahepatic cholestasis from a Chinese family with ARC syndrome. All 23 coding exons and flanking introns of the VPS33B gene were amplified and sequenced using peripheral lymphocyte genomic DNA of the patient and her parents. Genetic testing revealed two novel mutations (c.1033delA and c.1567C>T) in the VPS33B gene. The patient is a compound heterozygote and her parents were heterozygous for each of the mutations.展开更多
Grass carp plays an important role in small-scale aquaculture in Vietnam. However, a severe disease, known in Vietnam as "Red Spot Disease", is causing significant economic loss in grass carp aquaculture. In...Grass carp plays an important role in small-scale aquaculture in Vietnam. However, a severe disease, known in Vietnam as "Red Spot Disease", is causing significant economic loss in grass carp aquaculture. In this study, the tissue samples isolated from the grass carp with Red Spot Disease in Vietnam are investigated and comparied with the control GCHV isolated in China by experimental infection, culture cell infection, serological cross reactivity, and RT-PCR amplification. Infected grass carp exhibits hemorrhage symptoms about 5 days after experimental injection with GCHV-V (Vietnam) strain. The symptoms and lethality induced by the GCHV-V strain are identical to that induced by the Chinese GCHV-9014 strain. The Chinese GCHV-873 strain induces typical cytopathogenic effects in 4 cell lines, such as CIK, CAB, FHM and GCO, from the 6 fish cell lines examined. No cytopathogenic effects are observed in all the 6 examined cell lines, including CAB, FHM, CIK, EPC, CCO and GCO, infected by the GCHV-V strain and GCHV-9014 strain. Immunodiffusion assays demonstrate an obvious cross-reactivity among three GCHV strains. Precipitin lines are clearly observed not only between the anti-GCHV-873 serum and the two strains GCHV-873 and GCHV-9014, but also between the anti-GCHV-873 serum and the GCHV-V strain. GCHV can be detected by immunodiffusion assays after three generations of blind propagations in the cell lines inoculated by GCHV-V strain. This implicates that GCHV-V viruses have been replicated and amplified despite there being no cytopathogenic effects observed in these examined cell lines. Three genome segments of GCHV, including S8, S9 and S10, are amplified by three sets of PCR primers designed according to the segment sequences published recently. The Q8fp and Q8rp primer set specific for genome segment S8 amplifies a 955 bp fragment from the extracted sample of diseased fish with Red Spot Disease, and the fragment size is identical to that amplified by the same primer set from control GCHV-873 strain. Simultaneously, the Q9fp and Q9rp primer set specific for genome segment S9 generates a same 635 bp product, and the Q10fp and Q10rp primer set specific for genome segment S10 produces a same 697 bp fragment from both template samples of diseased fish with Red Spot Disease and control GCHV-873 strain. The RT-PCR amplification and corresponding size comparison data indicate that the three GCHV-V genome segments extracted from the diseased grass carp with Red Spot Disease in Vietnam should be identical to that in control GCHV-873 strain from China. The data confirm that the causative agent of grass carp Red Spot Disease in Vietnam is a virus, and the virus is closely similar to GCHV strain in China.展开更多
Eel family is a huge one, in which many kinds of eelsespecially some migratory eels, bear strong resemblance toeach other, and are therefOre difficult to be identified. Inthis study 29 random primers were used to make...Eel family is a huge one, in which many kinds of eelsespecially some migratory eels, bear strong resemblance toeach other, and are therefOre difficult to be identified. Inthis study 29 random primers were used to make RAPDana1ysis for Japanese ee1 (Angui11ajaponica). European eel(Anguilla anguilla) and Pike eel (AIllrae1lesox cinereus).And totally 299 fragments were counted. Shared or spe-cific fragments were counted alld ge11etic similarity or ge-netic distance were calculated. The genetic similarity be-tween Japanese eel alld Pike eel is 0.68 and the geneticdistance between them is 0.32; those between Europeaneel and Pike eel are 0.72 and 0.28 respectively, and be-tween Japanese eel and Ellropean eel are 0.74 and 0.25 re-spectively. The method has been shown to be suitable tomolecular identification of eels. It provides an a1ternativeapproach to determine the relatiollship between species.展开更多
A new ionic conductor La2-xNdxMo2O9 (x=0.0-2.0) has been synthesized by wet- chemistry method. The precursors and the resultant oxide powders were characterized by DTA/TG, DSC, XRD and XPS techniques. Effect of subs...A new ionic conductor La2-xNdxMo2O9 (x=0.0-2.0) has been synthesized by wet- chemistry method. The precursors and the resultant oxide powders were characterized by DTA/TG, DSC, XRD and XPS techniques. Effect of substituting Nd for La reveals that the phase transition which occurs in La2Mo2O9 around 565℃ disappears when x>0.2. And the maximum amount of Nd stabilized the high temperature phase of βLa2Mo2O9 from cubic to tetragonal is about x =1.6. The measurements of impedance spectroscopy indicate that the ionic conductivity becomes considerably higher in comparison to that of La2Mo2O9.展开更多
大模型作为人工智能领域的重要突破,展现出跨任务、跨模态的卓越泛化能力.尽管其在通用场景中表现出色,但在多样化领域中,其技术与应用拓展仍有待深入研究.为推动相关研究的发展,SCIENCE CHINA Information Sciences于2025年68卷第6期...大模型作为人工智能领域的重要突破,展现出跨任务、跨模态的卓越泛化能力.尽管其在通用场景中表现出色,但在多样化领域中,其技术与应用拓展仍有待深入研究.为推动相关研究的发展,SCIENCE CHINA Information Sciences于2025年68卷第6期组织出版了“大模型使能技术与前沿应用”专题(Special Topic:Enabling Techniques and Cutting-Edge Applications of Foundation Models),共收录10篇具有创新性与前瞻性的研究成果.Cui等在“Dynamic prompt allocation and tuning for continual test-time adaptation”中针对大模型在跨域测试自适应(test-time adaptation)中面临的灾难性遗忘问题。展开更多
基金Supported by National Natural Science Foundation of China,No.81070281
文摘Arthrogryposis, renal dysfunction and cholestasis (ARC) syndrome is a rare genetic disorder and has not been described in China. We present a female infant with neonatal intrahepatic cholestasis from a Chinese family with ARC syndrome. All 23 coding exons and flanking introns of the VPS33B gene were amplified and sequenced using peripheral lymphocyte genomic DNA of the patient and her parents. Genetic testing revealed two novel mutations (c.1033delA and c.1567C>T) in the VPS33B gene. The patient is a compound heterozygote and her parents were heterozygous for each of the mutations.
基金This research was supported by National 863 High Technology Research Foundation of China(2002AA62601)National Natural Science Foundation of China(30170726)+1 种基金the Project of Chinese Academy of Sciences(KSCXZ-SW-302)the Innovation Project of the Institute of Hydrobiology,Chinese Academy of Sciences.
文摘Grass carp plays an important role in small-scale aquaculture in Vietnam. However, a severe disease, known in Vietnam as "Red Spot Disease", is causing significant economic loss in grass carp aquaculture. In this study, the tissue samples isolated from the grass carp with Red Spot Disease in Vietnam are investigated and comparied with the control GCHV isolated in China by experimental infection, culture cell infection, serological cross reactivity, and RT-PCR amplification. Infected grass carp exhibits hemorrhage symptoms about 5 days after experimental injection with GCHV-V (Vietnam) strain. The symptoms and lethality induced by the GCHV-V strain are identical to that induced by the Chinese GCHV-9014 strain. The Chinese GCHV-873 strain induces typical cytopathogenic effects in 4 cell lines, such as CIK, CAB, FHM and GCO, from the 6 fish cell lines examined. No cytopathogenic effects are observed in all the 6 examined cell lines, including CAB, FHM, CIK, EPC, CCO and GCO, infected by the GCHV-V strain and GCHV-9014 strain. Immunodiffusion assays demonstrate an obvious cross-reactivity among three GCHV strains. Precipitin lines are clearly observed not only between the anti-GCHV-873 serum and the two strains GCHV-873 and GCHV-9014, but also between the anti-GCHV-873 serum and the GCHV-V strain. GCHV can be detected by immunodiffusion assays after three generations of blind propagations in the cell lines inoculated by GCHV-V strain. This implicates that GCHV-V viruses have been replicated and amplified despite there being no cytopathogenic effects observed in these examined cell lines. Three genome segments of GCHV, including S8, S9 and S10, are amplified by three sets of PCR primers designed according to the segment sequences published recently. The Q8fp and Q8rp primer set specific for genome segment S8 amplifies a 955 bp fragment from the extracted sample of diseased fish with Red Spot Disease, and the fragment size is identical to that amplified by the same primer set from control GCHV-873 strain. Simultaneously, the Q9fp and Q9rp primer set specific for genome segment S9 generates a same 635 bp product, and the Q10fp and Q10rp primer set specific for genome segment S10 produces a same 697 bp fragment from both template samples of diseased fish with Red Spot Disease and control GCHV-873 strain. The RT-PCR amplification and corresponding size comparison data indicate that the three GCHV-V genome segments extracted from the diseased grass carp with Red Spot Disease in Vietnam should be identical to that in control GCHV-873 strain from China. The data confirm that the causative agent of grass carp Red Spot Disease in Vietnam is a virus, and the virus is closely similar to GCHV strain in China.
文摘Eel family is a huge one, in which many kinds of eelsespecially some migratory eels, bear strong resemblance toeach other, and are therefOre difficult to be identified. Inthis study 29 random primers were used to make RAPDana1ysis for Japanese ee1 (Angui11ajaponica). European eel(Anguilla anguilla) and Pike eel (AIllrae1lesox cinereus).And totally 299 fragments were counted. Shared or spe-cific fragments were counted alld ge11etic similarity or ge-netic distance were calculated. The genetic similarity be-tween Japanese eel alld Pike eel is 0.68 and the geneticdistance between them is 0.32; those between Europeaneel and Pike eel are 0.72 and 0.28 respectively, and be-tween Japanese eel and Ellropean eel are 0.74 and 0.25 re-spectively. The method has been shown to be suitable tomolecular identification of eels. It provides an a1ternativeapproach to determine the relatiollship between species.
基金supported by the State Key Program of Basic Research (G1998061311) and the National Natural Science Foundation of China (29831010).
文摘A new ionic conductor La2-xNdxMo2O9 (x=0.0-2.0) has been synthesized by wet- chemistry method. The precursors and the resultant oxide powders were characterized by DTA/TG, DSC, XRD and XPS techniques. Effect of substituting Nd for La reveals that the phase transition which occurs in La2Mo2O9 around 565℃ disappears when x>0.2. And the maximum amount of Nd stabilized the high temperature phase of βLa2Mo2O9 from cubic to tetragonal is about x =1.6. The measurements of impedance spectroscopy indicate that the ionic conductivity becomes considerably higher in comparison to that of La2Mo2O9.
文摘大模型作为人工智能领域的重要突破,展现出跨任务、跨模态的卓越泛化能力.尽管其在通用场景中表现出色,但在多样化领域中,其技术与应用拓展仍有待深入研究.为推动相关研究的发展,SCIENCE CHINA Information Sciences于2025年68卷第6期组织出版了“大模型使能技术与前沿应用”专题(Special Topic:Enabling Techniques and Cutting-Edge Applications of Foundation Models),共收录10篇具有创新性与前瞻性的研究成果.Cui等在“Dynamic prompt allocation and tuning for continual test-time adaptation”中针对大模型在跨域测试自适应(test-time adaptation)中面临的灾难性遗忘问题。
