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Congenital disorder of glycosylation caused by mutation of ATP6AP1 gene (c.1036G>A) in a Chinese infant: A case report

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World Journal of Clinical Cases 2021年第26期9卷 7876-7885页

作者： Xia Yang Zi-Li Lv Qing Tang Xiu-Qi Chen Li Huang Mei-Xiong Yang lian-cheng lan Qing-Wen Shan Department of Pediatrics The First Affiliated Hospital of Guangxi Medical UniversityNanning 530021Guangxi Zhuang Autonomous RegionChina Department of Pathology The First Affiliated Hospital of Guangxi Medical UniversityNanning 530021Guangxi Zhuang Autonomous RegionChina

BACKGROUND The ATP6AP1 gene coding for the accessory protein Ac45 of the vacuolar-type adenosine triphosphatases(V-ATPase)is located on chromosome *** in certain subunits or accessory subunits of the V-ATPase can lead to congenital disorders of glycosylation(CDG).CDG is a group of metabolic disorders in which defective protein and lipid glycosylation processes affect multiple tissues and ***,the clinical presentation of patients with ATP6AP1-CDG varies *** this report,we present a case of ATP6AP1-CDG in a Chinese infant,with clinical features and *** SUMMARY An 8-mo-old boy was admitted to our hospital because unexplained hepatosplenomegaly and elevated transaminases that had been noted while he was being treated for a cough at a local hospital.A post-admission examination at our hospital revealed abnormalities in the infant’s liver,brain,and immune ***-based whole exome gene analysis identified a hemizygous pathogenic mutation c.1036G>A(p.E346K)in exon 9 of the ATP6AP1 *** variant of the ATP6AP1 gene has not been reported in East Asian countries until *** Based on the infant’s clinical manifestations and the results of genetic detection,he was clearly diagnosed with *** clinical manifestations of children with CDG vary *** testing analysis helps in the clinical diagnosis of children with CDG.

关键词： Congenital disorders of glycosylation ATP6AP1 mutation Hepatopathy Immunodeficiency Cognitive impairment Case report

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Evaluation of surface latent heat and sensible heat fluxes from ERA-5,GLDAS,and MODIS on different underlying surfaces in the Tibetan Plateau

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Journal of Mountain Science 2025年第1期22卷 230-245页

作者： lan Xincan YIN Yongsheng TANG Jiale lian Yuanyuan ZHAO Fang WANG Yumeng ZHENG Zhixian College of Geography and Environmental Science Henan UniversityKaifeng 475004China Institute of Mountain Hazards and Environment Chinese Academy of SciencesChengdu 610299China Key Laboratory of Geospatial Technology for the Middle and Lower Yellow River Regions(Henan University) Ministry of EducationKaifeng 475004China

Surface-latent heat(LE)and sensible heat(SH)fluxes play a pivotal role in governing hydrological,biological,geochemical,and ecological processes on the land surface in the Tibetan ***,to accurately assess and understand the spatial distribution of LE and SH fluxes across different underlying surfaces,it is crucial to verify the validity and reliability of ERA-5,GLDAS,and MODIS data against ground measurements obtained from the Flux Net micrometeorological tower *** study analyzed the spatial patterns of LE and SH over the Tibetan Plateau using data from ERA-5,GLDAS,and *** results were compared with ground measurements from Flux Net tower observations on different underlying surfaces,and five statistical parameters(Pearson's r,LR slope,RMSE,MBE,and MAE)were used to validate the *** results showed that:(1)MODIS LE data and ERA-5 SH data exhibited the closest agreement with ground observations,as indicated by their lowest root mean square error and mean bias area values.(2)The accuracy of ERA-5 SH was the highest in meadows and steppes,while GLDAS SH performed optimally in ***,MODIS LE consistently outperformed the other datasets across all vegetation types.(3)The spatial distribution of LE and SH displayed considerable heterogeneity,contingent upon the specific data sources and underlying ***,there was a contrasting trend between GLDAS and ERA-5,as well as MODIS,in terms of SH distribution in the *** shrublands and meadows,MODIS SH and LE exhibited more pronounced changes than ERA-5 and ***,ERA-5 SH demonstrated the opposite variation in meadow and steppe regions compared to GLDAS and MODIS.

关键词： FluxNet ERA-5 GLDAS MODIS Latent and sensible heat flux(LE and SH) Tibetan Plateau

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Distribution, characteristics, metallogenic processes and prospecting potential of terrestrial brine-type lithium deposits in the world and lithium demand situation

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China Geology 2025年第1期8卷 1-25页

作者： Dian-he Liu cheng-lin Liu Chun-lian Wang Xiao-can Yu MNR Key Laboratory of Metallogeny and Mineral Assessment Institute of Mineral ResourcesChinese Academy of Geological SciencesBeijing 100037China Peking University School of Earth and Space SciencesBeijing 100871China School of Earth Resources China University of Geosciences(Wuhan)Wuhan 430074China Institute of Geology Chinese Academy of Geological SciencesBeijing 100037China

In response to the rise of the energy storage industries such as new energy vehicles and the wide application of lithium in various fields worldwide,the global demand for lithium resources has been in explosive *** order to further comprehensively understand the global supply and demand pattern,development and utilization status,genesis of ore deposits and other characteristics of lithium resources,based on the achievements of many researchers at home and abroad,this paper systematically summarized the lithium supply and demand situation,resource endowment,deposit classification and distribution,typical geological characteristics,metallogenic factors and metallogenic regularity of terrestrial brine-type lithium deposits which are the main types of development and utilization all over the *** review shows that brine-type lithium resource and(or)reserves in the plateau salt lakes are huge and play an important *** addition,the mineralization potential of the underground brine-type lithium deposit is broad *** potential resources of underground brines are enormous,and the geothermal spring water type is also worthy of *** lithium deposits are mainly controlled by the subduction and collision of regional plate tectonics,arid climate and provenance *** of the scientific research on underground brines in the future is expected to provide another significant support for the global demand for lithium resources.

关键词： Brine in plateau salt lake Underground brine Geothermal spring brine Terrestrial brine classification Lithium migration mechanism Brine genesis Mineral exploration engineering Lithium enrich mechanism

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Receiver-device-independent quantum secure direct communication

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Science China(Physics,Mechanics & Astronomy) 2025年

作者： cheng Liu cheng Zhang Shi-Pu Gu Xing-Fu Wang lan Zhou Yu-Bo Sheng College of Science Nanjing University of Posts and Telecommunications College of Electronic and Optical Engineering College of Flexible Electronics (Future Technology)Nanjing University of Posts and Telecommunications

Quantum secure direct communication (QSDC) enables the sender to directly transmit secure messages to the receiver via a quantum channel without the need for ***-independent (DI) and measurement-device-independent (MDI) QSDC protocols theoretically enhance the practical security of ***,DI QSDC requires extremely high global detection efficiency and has a limited secure communication *** DI and MDI QSDC protocols depend on high-quality *** entanglement sources,which generate entangled photon pairs with low efficiency,significantly reduce their practical communication *** this paper,we propose a single-photon-based receiver-device-independent (RDI) QSDC *** protocol relies solely on the practical single-photon source,which is nearly on-demand with current technology,and treats all receiving devices on both ends of the communication as“black boxes”.The security of the message is ensured only through the observed *** also develop a numerical method to simulate the protocol’s performance under practical noisy communication *** RDI QSDC protocol provides the same security level as MDI *** to DI and MDI QSDC,RDI QSDC has several ***,it uses single-photon sources and single-photon measurements,which allow it to achieve practical communication efficiency approximately 3415 times greater than that of DI QSDC,while being easier to *** entire protocol is feasible with current ***,it offers higher robustness to photon loss and better noise tolerance than DI QSDC,enabling a secure communication distance approximately 26 times greater than that of DI *** on these advantages,the RDI QSDC protocol presents a promising approach for achieving highly secure and efficient QSDC in the near future.

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Serum ECM1 is a promising biomarker for staging and monitoring fibrosis in patients with chronic hepatitis B

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Science China(Life Sciences) 2025年第2期68卷 431-440页

作者： lian Liu Danyan Zhang Rong Fan Shipeng cheng Jichao Yang Liyan Ma Zhiyang Ling Yaguang Zhang Jinlin Hou Xiaomei Wang Bing Sun Junqi Niu Shanghai Institute of Biochemistry and Cell Biology Centre for Excellence in Molecular Cell ScienceChinese Academy of SciencesUniversity of Chinese Academy of SciencesShanghai 200031China School of Life Science and Technology ShanghaiTech UniversityShanghai 201210China Department of Infectious Disease Nanfang HospitalSouthern Medical UniversityGuangzhou 510515China Med-X Institute Centre for Immunological and Metabolic DiseasesThe First Affiliated Hospital of Xi’an Jiaotong UniversityXi’an Jiaotong UniversityXi’an 710061China Hepatology Department Centre of Infectious Diseases and Pathogen BiologyFirst Hospital of Jilin UniversityChangchun 130021China

It is critical to assess the extent and progression of liver fibrosis for patients to receive suitable treatments,but its diagnostic methods remain *** matrix protein 1(ECM1)has previously been reported to be a key factor in the induction and progression of liver ***,little is known about the use of ECM1 as a biomarker to evaluate *** a CCl_(4)-induced mouse model of liver fibrosis,the present study demonstrated that ECM1 decreased with gradually increasing *** biopsy as a reference,the serum ECM1 levels decreased with increasing fibrosis stage in 247 patients with liver fibrosis,but there were no significant changes between fibrosis stage 2 and stage 0–*** improve the performance of ECM1,age,platelet count,and ECM1 concentration were combined to calculate an EPA(ECM1-platelet-age)score(ranging from 0 to 10).The areas under the receiver operating characteristic curve of the EPA scores for the detection of F≥2,F≥3,and F4 were 0.6801,0.7377,and 0.8083,respectively,which showed a comparable or significantly greater diagnostic performance for assessing fibrosis than that of the AST/ALT ratio,APRI score,or FIB-4 *** HBV patients following antiviral treatment,the dynamics of the EPA score depended on the status of liver fibrosis *** accuracy of the EPA score in predicting fibrosis regression and progression was 66.00%and 71.43%,respectively,while that of the LSM,another useful method for monitoring hepatic fibrosis changes during treatment,was only 52.00%and 7.14%,*** with healthy controls,there were lower levels of serum ECM1 in HBV patients and individuals with HCV infection,MAFLD,ALD,PBC,and *** findings suggested that individuals with reduced ECM1 levels may have a risk of developing liver injury,and further examinations or medical care are *** conclusion,the ECM1-containing EPA score is a valuable noninvasive test for staging fibrosis and predicting the progression of liv

关键词： ECM1 EPA score noninvasive biomarker dynamic liver fibrosis injury

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The circadian clock affects starvation resistance through the pentose phosphate pathway in silkworm,Bombyx mori

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Insect Science 2025年第1期32卷 55-68页

作者： Tai-Ming Dai Jian-Feng Qiu cheng Luo Wen-Zhao Cui Kai Liu Jiang-lan Li Ruji Peng Yang-Hu Sima Shi-Qing Xu School of Life Sciences Suzhou Medical College of Soochow UniversitySuzhouJiangsu ProvinceChina Institute of Agricultural Biotechnology&Ecology(IABE) Soochow UniversitySuzhouJiangsu ProvinceChina

Disruption of the circadian clock can affect starvation resistance,but the molecular mechanism is still ***,we found that starvation resistance was significantly reduced in the core gene BmPer deficient mutant silkworms(Per−/−),but the mutant's starvation resistance increased with larval *** natural physiological conditions,the weight of mutant 5th instar larvae was significantly increased compared to wild type,and the accumulation ability of triglycerides and glycogen in the fat bodies was ***,under starvation conditions,the weight consumption of mutant larvae was increased and cholesterol utilization was *** analysis showed that beta-oxidation was significantly upregulated under starvation conditions,fatty acid synthesis was inhibited,and the expression levels of genes related to mitochondrial function were significantly *** investigations revealed that the redox balance,which is closely related to mitochondrial metabolism,was altered in the fat bodies,the antioxidant level was increased,and the pentose phosphate pathway,the source of reducing power in cells,was *** findings suggest that one of the reasons for the increased energy burden observed in mutants is the need to maintain a more robust redox balance in metabolic *** necessitates the diversion of more glucose into the pentose phosphate pathway to ensure an adequate supply of reducing power.

关键词： Bombyx mori circadian clock oxidative stress pentose phosphate pathway starvationresistance

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Laparoscopic liver resection utilizing the ventral avascular area of the inferior vena cava:A retrospective cohort study

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World Journal of Gastroenterology 2025年第1期31卷 42-57页

作者： Kun Huang Zhu Chen Heng Xiao Hai-Yang Hu Xing-Yu Chen cheng-You Du Xiang lan Department of Hepatobiliary Surgery The First Affiliated Hospital of Chongqing Medical UniversityChongqing 400000China Department of General Surgery Mianyang Hospital of Traditional Chinese MedicineMianyang 621000Sichuan ProvinceChina

BACKGROUND Laparoscopic liver resection(LLR)can be challenging due to the difficulty of establishing a retrohepatic tunnel under *** the third hepatic hilum before parenchymal transection often leads to significant liver mobilization,tumor compression,and bleeding from the short hepatic veins(SHVs).This study introduces a novel technique utilizing the ventral avascular area of the inferior vena cava(IVC),allowing SHVs to be addressed after parenchymal transection,thereby reducing surgical complexity and improving outcomes in in situ *** To introduce and evaluate a novel LLR technique using the ventral avascular area of the IVC and compare its short-term outcomes with conventional *** The clinical cohort data of patients with pathologically confirmed hepatocellular carcinoma or intrahepatic cholangiocarcinoma who underwent conventional LLR and novel LLR between July 2021 and July 2023 at the First Affiliated Hospital of Chongqing Medical University were retrospectively *** novel LLR,we initially separated the caudate lobe from the IVC using dissecting forceps along the ventral avascular area of the ***,we transected the parenchyma of the left and right caudate lobes from the caudal side to the cephalic side using the avascular area as a ***,we addressed the SHVs and finally dissected the root of the right hepatic vein or left hepatic *** short-term postoperative outcomes and oncological results of the two approaches were evaluated and *** A total of 256 patients were included,with 150(58.59%)undergoing conventional LLR and 106(41.41%)undergoing novel *** novel technique resulted in significantly larger tumor resections(6.47±2.96 cm vs 4.01±2.33 cm,P<0.001),shorter operative times(199.57±60.37 minutes vs 262.33±83.90 minutes,P<0.001),less intraoperative blood loss(206.92±37.09 mL vs 363.34±131.27 mL,P<0.001),and greater resection volume(345.11±31.40 mL vs 264.38±31.98 mL,P<0.001)comp

关键词： Laparoscopic liver resection Inferior vena cava Retrohepatic tunnel Short hepatic veins Complex liver tumors Intraoperative bleeding control

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Penile metastasis from colon cancer with BRAF_(V600E)mutation treated with BRAF/MEK-targeted therapy plus cetuximab:A case report

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World Journal of Gastrointestinal Oncology 2025年第3期17卷 402-409页

作者： Jun-Chen Wu Hua-Xi cheng Qiu-Sheng lan He-Yang Xu Yu-Jie Zeng Wei Lai Zhong-Hua Chu Department of Gastrointestinal Surgery Sun Yat-sen Memorial HospitalSun Yat-sen UniversityGuangzhou 510120Guangdong ProvinceChina

BACKGROUND The incidence of colon cancer has been progressively increasing over time,whereas penile metastasis of colon cancer has remained exceedingly *** the prognosis for colon cancer with BRAF_(V600E)mutation is relatively unfavorable,further exploration and investigation are still required to develop treatment strategies for such rare *** SUMMARY About one year after surgery and chemotherapy,a 50-year-old patient with sigmoid colon cancer developed a mass at the base of the patient's penis,accompanied by severe tenderness and pain during *** disease progression,multiple metastatic nodules also emerged in other regions of the penis,including the coronal *** nodules located in the coronal sulcus were excised for histopathological *** histopathological findings revealed that the nodules were metastases originating from the sigmoid colon cancer,with a BRAF V600E mutation *** prompted a modification of the therapy regimen of cetuximab,dabrafenib and trametinib,which effectively held back the progression of penile metastasis in the *** Combining the BRAF/MEK-targeted therapy with cetuximab demonstrates a favorable therapeutic response in BRAF_(V600E)-mutated colon cancer with penile metastasis.

关键词： Colon cancer Penile metastasis BRAF_(V600E)mutation Targeted therapy Case report

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Ni-catalyzed cross-electrophile alkyl-alkyl coupling reactions

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Science China Chemistry 2025年

作者： Li-Xu You lan Tian Chun-Ling Guo Shun-Xi Li Yu-cheng Liu Yu-Long Li Wei Shu College of Chemistry and Environmental Engineering Key Laboratory of Green Catalysis of Higher Education Institutes of Sichuan Sichuan University of Science and Engineering Guangming Advanced Research Institute Shenzhen Grubbs Institute Department of Chemistry and Guangdong Provincial Key Laboratory of Catalysis Southern University of Science and Technology

Introducing saturated carbon-carbon bonds into organic molecules significantly increases the three-dimensional configuration,thus altering the physical and chemical properties as well as the functions of molecules. Transition-metal-catalyzed carboncarbon cross-couplings represent one of the most straightforward, efficient and modular alternatives to build saturation in a single step. However, the efficient construction of alkyl-alkyl bonds remains a formidable challenge due to the precise manipulation of multiple alkyl metallic intermediates as well as associated competitive side reactions and selectivity control. Over the past years, Ni-catalyzed cross-coupling under reductive conditions has gained more and more attention for alkyl-alkyl crosscoupling partially attributing to the low-valent state of metal centers under reducing conditions, which suppresses the competitive β-hydride elimination process, thus facilitating the formation of alkyl-alkyl bonds. In this review, the initiative and progress of Ni-catalyzed cross-electrophile alkyl-alkyl cross-coupling, including the efforts in racemic and enantioselective formation of alkyl-alkyl bonds, have been summarized and discussed. Moreover, the challenges and further opportunities in this area have also been discussed.

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Influence of laser shock peening on microstructure and high-temperature oxidation resistance of Ti45Al8Nb alloy fabricated via laser melting deposition

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Transactions of Nonferrous Metals Society of China 2025年第1期35卷 157-168页

作者： Lu-lu JIANG liang lan cheng-yan BAI Ru-yi XIN Shuang GAO Hao-yu WANG Bo HE Chao-yue CHEN Guo-xin LU Research Center of High-Temperature Alloy Precision Forming School of Materials Science and EngineeringShanghai University of Engineering ScienceShanghai 201620China State Key Laboratory of Advanced Special Steels School of Materials Science and EngineeringShanghai UniversityShanghai 200444China School of Materials Science and Engineering Shandong UniversityJinan 250061China

Laser shock peening(LSP)was used to enhance the high-temperature oxidation resistance of laser melting deposited Ti45Al8Nb *** microstructure and high-temperature oxidation behavior of the as-deposited Ti45Al8Nb alloy before and after LSP were investigated by scanning electron microscopy,X-ray diffraction,and electron backscatter *** results indicated that the rate of mass gain in the as-deposited sample after LSP exhibited a decrease when exposed to an oxidation temperature of 900℃,implying that LSP-treated samples exhibited superior oxidation resistance at high temperatures.A gradient structure with a fine-grain layer,a deformed-grain layer,and a coarse-grain layer was formed in the LSP-treated sample,which facilitated the diffusion of the Al atom during oxidation,leading to the formation of a dense Al_(2)O_(3)layer on the *** mechanism of improvement in the oxidation resistance of the as-deposited Ti45Al8Nb alloy via LSP was discussed.

关键词： TiAl alloy laser melting deposition laser shock peening additive manufacturing oxidation resistance

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