Certain amino acids changes in the human Na^(+)/K^(+)-ATPase pump,ATPase Na^(+)/K^(+)transporting subunit alpha 1(ATP1A1),cause Charcot-Marie-Tooth disease type 2(CMT2)disease and refractory seizures.To develop in viv...Certain amino acids changes in the human Na^(+)/K^(+)-ATPase pump,ATPase Na^(+)/K^(+)transporting subunit alpha 1(ATP1A1),cause Charcot-Marie-Tooth disease type 2(CMT2)disease and refractory seizures.To develop in vivo models to study the role of Na^(+)/K^(+)-ATPase in these diseases,we modified the Drosophila gene homolog,Atpα,to mimic the human ATP1A1 gene mutations that cause CMT2.Mutations located within the helical linker region of human ATP1A1(I592T,A597T,P600T,and D601F)were simultaneously introduced into endogenous Drosophila Atpαby CRISPR/Cas9-mediated genome editing,generating the Atpα^(TTTF)model.In addition,the same strategy was used to generate the corresponding single point mutations in flies(Atpα^(I571T),Atpα^(A576T),Atpα^(P579T),and Atpα^(D580F)).Moreover,a deletion mutation(Atpα^(mut))that causes premature termination of translation was generated as a positive control.Of these alleles,we found two that could be maintained as homozygotes(Atpα^(I571T)and Atpα^(P579T)).Three alleles(Atpα^(A576T),Atpα^(P579)and Atpα^(D580F))can form heterozygotes with the Atpαmut allele.We found that the Atpαallele carrying these CMT2-associated mutations showed differential phenotypes in Drosophila.Flies heterozygous for Atpα^(TTTF)mutations have motor performance defects,a reduced lifespan,seizures,and an abnormal neuronal morphology.These Drosophila models will provide a new platform for studying the function and regulation of the sodium-potassium pump.展开更多
A designed Mg_(88.7)Ni_(6.3)Y_(5)hydrogen storage alloy containing 14H type LPSO(long-period stacking ordered)and ternary eutectic structure was prepared by regulating the alloy composition and casting.The hydrogen st...A designed Mg_(88.7)Ni_(6.3)Y_(5)hydrogen storage alloy containing 14H type LPSO(long-period stacking ordered)and ternary eutectic structure was prepared by regulating the alloy composition and casting.The hydrogen storage performance of the alloy was improved by adding nano-flower-like TiO_(2)@C catalyst.The decomposition of the LPSO structure during hydrogenation led to the formation of plenty of nanocrystals which provided abundant interphase boundaries and activation sites.The nanoscale TiO_(2)@C catalyst was uniformly dispersed on the surface of alloy particles,and the"hydrogen overflow''effect of TiO_(2)@C accelerated the dissociation and diffusion of hydrogen on the surface of the alloy particles.As a result,the in-situ endogenous nanocrystals of the LPSO structure decomposition and the externally added flower-like TiO_(2)@C catalyst uniformly dispersed on the surface of the nanoparticles played a synergistic catalytic role in improving the hydrogen storage performance of the Mg-based alloy.With the addition of the TiO_(2)@C catalyst,the beginning hydrogen desorption temperature was reduced to 200℃.Furthermore,the saturated hydrogen absorption capacity of the sample was 5.32 wt.%,and it reached 4.25 wt.%H_(2) in 1 min at 200℃and 30 bar.展开更多
直接焚烧核燃料后处理过程中产生的磷酸三丁酯(TBP)和煤油(OK)有机废液会产生磷酸,会对设备造成一定程度的腐蚀,因此在采用热解焚烧工艺处理该废液时,需掺入合适比例的中和剂和表面活性剂等添加剂,将废液配制成均匀稳定的悬浮液。中和...直接焚烧核燃料后处理过程中产生的磷酸三丁酯(TBP)和煤油(OK)有机废液会产生磷酸,会对设备造成一定程度的腐蚀,因此在采用热解焚烧工艺处理该废液时,需掺入合适比例的中和剂和表面活性剂等添加剂,将废液配制成均匀稳定的悬浮液。中和剂在热解过程中可以固定P2O5,避免磷酸生成后对设备的腐蚀。本文通过不同单因素实验研究以Ca(OH)_(2)和Mg(OH)_(2)为中和剂对配制成的TBP/OK悬浮液性能的影响,为后处理厂热解焚烧系统提供新的悬浮液配方思路。试验结果显示:当TBP含量为30%和60%时,Mg(OH)_(2)在相同TBP含量下配制成的悬浮液的最终表面黏度远大于Ca(OH)_(2),Mg(OH)_(2)和Ca(OH)_(2)在30%TBP时的最终表面黏度分别为90.36 m Pa·s和43.42 mPa·s,在60%TBP时的最终表面黏度分别为95.24 m Pa·s和75.09 m Pa·s,同时Mg(OH)_(2)的乳化速度明显优于Ca(OH)_(2),并具有更好的流动性。以Mg(OH)_(2)为中和剂的悬浮液在不同核素和DBP含量下整体稳定性更好。展开更多
基金supported by the Natural Science Foundation of Fujian Province,No.2020J02027the National Natural Science Foundation of China,No.31970461the Foundation of NHC Key Laboratory of Technical Evaluation of Fertility Regulation for Non-human Primate,Fujian Maternity and Child Health Hospital,No.2022-NHP-05(all to WC).
文摘Certain amino acids changes in the human Na^(+)/K^(+)-ATPase pump,ATPase Na^(+)/K^(+)transporting subunit alpha 1(ATP1A1),cause Charcot-Marie-Tooth disease type 2(CMT2)disease and refractory seizures.To develop in vivo models to study the role of Na^(+)/K^(+)-ATPase in these diseases,we modified the Drosophila gene homolog,Atpα,to mimic the human ATP1A1 gene mutations that cause CMT2.Mutations located within the helical linker region of human ATP1A1(I592T,A597T,P600T,and D601F)were simultaneously introduced into endogenous Drosophila Atpαby CRISPR/Cas9-mediated genome editing,generating the Atpα^(TTTF)model.In addition,the same strategy was used to generate the corresponding single point mutations in flies(Atpα^(I571T),Atpα^(A576T),Atpα^(P579T),and Atpα^(D580F)).Moreover,a deletion mutation(Atpα^(mut))that causes premature termination of translation was generated as a positive control.Of these alleles,we found two that could be maintained as homozygotes(Atpα^(I571T)and Atpα^(P579T)).Three alleles(Atpα^(A576T),Atpα^(P579)and Atpα^(D580F))can form heterozygotes with the Atpαmut allele.We found that the Atpαallele carrying these CMT2-associated mutations showed differential phenotypes in Drosophila.Flies heterozygous for Atpα^(TTTF)mutations have motor performance defects,a reduced lifespan,seizures,and an abnormal neuronal morphology.These Drosophila models will provide a new platform for studying the function and regulation of the sodium-potassium pump.
基金partially supported by the National Key R&D Program of China(No.2020YFA0406204)the National Natural Science Foundation of China(No.52201265)+1 种基金Shaanxi Province Key Project of Research and Development Plan,China(No.2023-YBGY-294,No.2023KXJ-060)the Doctoral Scientific Research Starting Foundation of Shaanxi University of Science and Technology,China(No.2016GBJ-02)。
文摘A designed Mg_(88.7)Ni_(6.3)Y_(5)hydrogen storage alloy containing 14H type LPSO(long-period stacking ordered)and ternary eutectic structure was prepared by regulating the alloy composition and casting.The hydrogen storage performance of the alloy was improved by adding nano-flower-like TiO_(2)@C catalyst.The decomposition of the LPSO structure during hydrogenation led to the formation of plenty of nanocrystals which provided abundant interphase boundaries and activation sites.The nanoscale TiO_(2)@C catalyst was uniformly dispersed on the surface of alloy particles,and the"hydrogen overflow''effect of TiO_(2)@C accelerated the dissociation and diffusion of hydrogen on the surface of the alloy particles.As a result,the in-situ endogenous nanocrystals of the LPSO structure decomposition and the externally added flower-like TiO_(2)@C catalyst uniformly dispersed on the surface of the nanoparticles played a synergistic catalytic role in improving the hydrogen storage performance of the Mg-based alloy.With the addition of the TiO_(2)@C catalyst,the beginning hydrogen desorption temperature was reduced to 200℃.Furthermore,the saturated hydrogen absorption capacity of the sample was 5.32 wt.%,and it reached 4.25 wt.%H_(2) in 1 min at 200℃and 30 bar.
文摘直接焚烧核燃料后处理过程中产生的磷酸三丁酯(TBP)和煤油(OK)有机废液会产生磷酸,会对设备造成一定程度的腐蚀,因此在采用热解焚烧工艺处理该废液时,需掺入合适比例的中和剂和表面活性剂等添加剂,将废液配制成均匀稳定的悬浮液。中和剂在热解过程中可以固定P2O5,避免磷酸生成后对设备的腐蚀。本文通过不同单因素实验研究以Ca(OH)_(2)和Mg(OH)_(2)为中和剂对配制成的TBP/OK悬浮液性能的影响,为后处理厂热解焚烧系统提供新的悬浮液配方思路。试验结果显示:当TBP含量为30%和60%时,Mg(OH)_(2)在相同TBP含量下配制成的悬浮液的最终表面黏度远大于Ca(OH)_(2),Mg(OH)_(2)和Ca(OH)_(2)在30%TBP时的最终表面黏度分别为90.36 m Pa·s和43.42 mPa·s,在60%TBP时的最终表面黏度分别为95.24 m Pa·s和75.09 m Pa·s,同时Mg(OH)_(2)的乳化速度明显优于Ca(OH)_(2),并具有更好的流动性。以Mg(OH)_(2)为中和剂的悬浮液在不同核素和DBP含量下整体稳定性更好。