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Updated clinical and glycomic features of mannosyl-oligosaccharide glucosidase deficiency:Two case reports 被引量:1
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作者 Kuerbanjiang Abuduxikuer Lei Wang +6 位作者 Lin Zou Cui-Yan Cao Long Yu Hong-Mei Guo Xin-Miao Liang Jian-She Wang Li Chen 《World Journal of Clinical Cases》 SCIE 2022年第21期7397-7408,共12页
BACKGROUND Mannosyl-oligosaccharide glucosidase(MOGS)deficiency is an extremely rare type of congenital disorder of glycosylation(CDG),with only 12 reported cases.Its clinical,genetic,and glycomic features are still e... BACKGROUND Mannosyl-oligosaccharide glucosidase(MOGS)deficiency is an extremely rare type of congenital disorder of glycosylation(CDG),with only 12 reported cases.Its clinical,genetic,and glycomic features are still expanding.Our aim is to update the novel clinical and glycosylation features of 2 previously reported patients with MOGS-CDG.CASE SUMMARY We collected comprehensive clinical information,and conducted the immunoglobulin G1 glycosylation assay using nano-electrospray ionization source quadruple time-of-flight mass spectrometry.Novel dysmorphic features included an enlarged tongue,forwardly rotated earlobes,a birth mark,overlapped toes,and abnormal fat distribution.Novel imaging findings included pericardial effusion,a deep interarytenoid groove,mild congenital subglottic stenosis,and laryngomalacia.Novel laboratory findings included peripheral leukocytosis with neutrophil predominance,elevated C-reactive protein and creatine kinase,dyslipidemia,coagulopathy,complement 3 and complement 4 deficiencies,decreased proportions of T lymphocytes and natural killer cells,and increased serum interleukin 6.Glycosylation studies showed a significant increase of hypermannosylated glycopeptides(Glc3Man7GlcNAc2/N2H10 and Man5GlcNAc2/N2H5)and hypersialylated glycopeptides.A compensatory glycosylation pathway leading to an increase in Man5GlcNAc2/N2H5 was indicated with the glycosylation profile.CONCLUSION We confirmed abnormal glycomics in 1 patient,expanding the clinical and glycomic spectrum of MOGS-CDG.We also postulated a compensatory glycosylation pathway,leading to a possible serum biomarker for future diagnosis. 展开更多
关键词 Mannosyl-oligosaccharide glucosidase MOGS-CDG Congenital disorder of glycosylation type IIb Mannosyl-oligosaccharide glucosidase gene Glycomics of IgG1 Case report
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Requirement of a Homolog of Glucosidase Ⅱ β-Subunit for EFR-Mediated Defense Signaling in Arabidopsis thaliana 被引量:2
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作者 Nina von Numers Mantas Survila +4 位作者 Markku Aalto Martine Batoux Pekka Heino E. Tapio Palva Jing Li 《Molecular Plant》 SCIE CAS CSCD 2010年第4期740-750,共11页
EFR is a plasma-membrane resident receptor responsible for recognition of microbial elongation factorTu (EF-Tu) and thus triggering plant innate immunity to fend off phytopathogens. Functional EFR must be subject to... EFR is a plasma-membrane resident receptor responsible for recognition of microbial elongation factorTu (EF-Tu) and thus triggering plant innate immunity to fend off phytopathogens. Functional EFR must be subject to the endoplasmic reticulum quality control (ERQC) machinery for the correct folding and proper assembly in order to reach its final destination. Genetic studies have demonstrated that ERD2b, a counterpart of the yeast or mammalian HDEL receptor ERD2 for retaining proteins in the endoplasmic reticulum (ER) lumen, is required for EFR function in plants (Li et al., 2009). In this study, we characterized the Arabidopsis glucosidase Ⅱ β--subunit via the H DEL motif against the non-redundant protein database. Data mining also revealed that the glucosidase Ⅱ β--subunit gene has a highly similar expression pattern to ERD2b and the other known ERQC components involved in EFR biogenesis. Importantly, the T-DNA insertion lines of the glucosidase Ⅱ β-subunit gene showed that EFR-controlled responses were substantially reduced or completely blocked in these mutants. The responses include seedling growth inhibition, induction of marker genes, MAP kinase activation, and callose deposition, trigged by peptide elf18, a full mimic of E F-Tu. Taken together, ourdata indicate a requirement of the glucosidase Ⅱ β-subunitfor EFR function. 展开更多
关键词 Arabidopsis thaliana glucosidase β-subunit endoplasmic reticulum quality control EFR innate immunity.
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High production of β-glucosidase from a marine Aspergillus niger immobilized on towel gourd vegetable sponges 被引量:2
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作者 Dong-Sheng Xue Jiang-Bo Wang Shan-Jing Yao 《Chinese Chemical Letters》 SCIE CAS CSCD 2015年第8期1011-1015,共5页
To produce β-glucosidase by consecutive batch fermentation, a marine Aspergillus niger was immobilized on a natural carrier, towel gourd vegetable sponges. The immobilized mycelia were 0.15 g/g carrier with the immob... To produce β-glucosidase by consecutive batch fermentation, a marine Aspergillus niger was immobilized on a natural carrier, towel gourd vegetable sponges. The immobilized mycelia were 0.15 g/g carrier with the immobilized biomass percentage of over 95%. The immobilized mycelia possessed the long durability(22.5 days). The maximum production occurred 1.5 day earlier by the immobilized mycelia than by the free mycelia. β-Glucosidase production of five consecutive batches was over 110 U/m L. At high salinity,the activity and stability of β-glucosidase from the marine A. niger increased remarkable. Immobilizing the marine A. niger on the novel natural carrier achieved the efficient production of β-glucosidase. 展开更多
关键词 glucosidase niger vegetable batch durability earlier salinity biomass reserved producing
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Inhibitory Activities on α-Glucosidase and Composition Analysis of Different Fractions of Ethanol Extracts from Actinidia chinensis Radix
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作者 NIE Changping YANG Jun +2 位作者 WU Dan WAN Luping LIANG Guangping 《Chemical Research in Chinese Universities》 SCIE CAS CSCD 2019年第5期823-829,共7页
Tlie inhibitory actions of ethyl acetate,n-butanol and water fractions from Actinidia chinensis Radix (A. chinensis Radix) ethanol extracts on glucosidase were investigated. We found that the inhibitory activity of di... Tlie inhibitory actions of ethyl acetate,n-butanol and water fractions from Actinidia chinensis Radix (A. chinensis Radix) ethanol extracts on glucosidase were investigated. We found that the inhibitory activity of different ethanol extract fractions on α-glucosidase is concentration-dependent. At the same concentration, the ethyl acetate and n-butaiiol fractions showed higher inliibitory activities on gglucosidase in comparison witli acarbose. We then determined the chemical constituents of tlie ethyl acetate and n-butanol fractions through ultra performance liquid cliromatography-triple-time of flight/mass spectrometry(UPLC-Triple-TOF/MS). Based on the mass spectrometry analysis and the databases Scitinder and Reaxys, 21 compoiinds(A1-A21) were identified from the ethyl acetate fraction. In addition, 11 of those compounds(A2, A3, A5, A6, A8, A10, A11, A13, A15, A19, A20) were identified for the first time from A. chinensis Radix. We also identified 14 compounds(A22-A32 and A4-A6) from tlie w-butanol fraction and compound A29 is a new natural product. From the n-butanol fraction, 7 additional compounds (A22, A25, A27, A28, A30-A32) were identified from A. chinensis Radix for the first time. 展开更多
关键词 ACTINIDIA chinensis RADIX Extract /glucosidase Ultra perfbnnance liquid chromatography-triple-time of flight/mass spectrometry(UPLC-Triple-TOF/MS)
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GLUCOSIDASE INHIBITORY ACTIVITY AND HPLC ANALYSIS OF THE FLAVONOIDS OBTAINED FROM IMPATIENS BALSAMINA L. OF DIFFERENT REGIONS
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作者 Meng Li Qian Li +1 位作者 Zhenghong Guo Yuqing Zhao 《World Journal of Traditional Chinese Medicine》 2015年第4期107-107,共1页
A simple and efficient HPLC method was developed for quality analysis of flavonoids from Impatiens balsamina L.(IBL)flowers,which were collected from Xingjiang,Anhui,Henan,and Hubei provinces in China.The flavonoids s... A simple and efficient HPLC method was developed for quality analysis of flavonoids from Impatiens balsamina L.(IBL)flowers,which were collected from Xingjiang,Anhui,Henan,and Hubei provinces in China.The flavonoids substances in IBL were determined by HPLC through methyl alcohol ultrasonic extraction.A mixture of 展开更多
关键词 IBL glucosidase INHIBITORY ACTIVITY AND HPLC ANALYSIS OF THE FLAVONOIDS OBTAINED FROM IMPATIENS BALSAMINA L OF DIFFERENT REGIONS
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Effects of tripchlorolide on the epididymides and testes of rats 被引量:10
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作者 Zuo-Peng WANG Zhi-Ping GU +4 位作者 Lin CAO Yang XU Gen-Di YOU Bai-Yong MAO Shao-Zhen QIAN Shanghai Institute of Materia Medical, Chinese Academy of Sciences, Shanghai 200031, China 《Asian Journal of Andrology》 SCIE CAS CSCD 1999年第3期121-125,共5页
Aim: To further evaluate the antifertility effects of tripchorolide, a derivative of triptolide produced at the extraction pro-cedure of Tripterygium wilfordii Hook. f., in male rats and to investigate its sites and p... Aim: To further evaluate the antifertility effects of tripchorolide, a derivative of triptolide produced at the extraction pro-cedure of Tripterygium wilfordii Hook. f., in male rats and to investigate its sites and possible mechanisms of action.Methods: In male rats, tripchlorolide was given by oral garage at a dose of 50 ug.kg~(-l).d~(-1) for 5 weeks, fertility wasassessed by mating tests, and biochemical indices and light microscopic observation of the epididymides and testes werealso performed. Results: Administration of tripchlorolide at 50 ugg.kg~(-l)-d~(-1) for 3 weeks did not influence the fertilityof male rats, but 5-week treatment rendered the rats infertile. The density and motility of spermatozoa collected fromcauda epididymides were reduced significantly. The epididymal weights, as well as the L-carnitine concentration and α-glucosidase content in the epididymal fluid were decreasd. There were no significant differences in α-glucosidase andacid phosphatase (ACP) in caput epididymal homogenates between the control and the experimental rats. Obvious mor-phological changes were observed in the epididymal spermatozoa, mainly including head and tail separation or acrosomecurving. Sloughed spermatids were found in the seminifeous and epididymal tubules. In tesficular homogenates,tripchlorolide had no influence on the lactate dehydrogenase-C_4 (LDH-C_4) and hyaluronidase activities. No apparentlesions were observed in the seminiferous and epididymal epithelium. Conclusion: At the dose level employed,tripchlorolide has a significant effect on the fertility in male rats and the primary sites of action may be spermatids and tes-ticular and epididymal spermatozoa. (Asian J Androl 1999 Sep ; 1: 121 - 125) 展开更多
关键词 TRIPCHLOROLIDE EPIDIDYMIS TESTIS carmitine glucosidases lactate dehydrogenase HYALURONIDASE
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血清AFU、LAP和GGT联合检测在早期原发性肝癌诊断中的应用与评价 被引量:10
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作者 隋菱 《中国实验诊断学》 2016年第7期1088-1089,共2页
我国是肝炎发病大国,肝癌发病率也呈逐年上升的趋势,是临床常见的疾病。据统计,每年大约有11万人因肝癌死亡。肝癌一般起病比较隐蔽,早期无典型的临床症状,临床上一旦确诊一般属于中、晚期。因此,如何早期诊断肝癌具有重要的意义。
关键词 肝癌发生 早期肝癌 原发性肝癌 AFU GGT LAP 影像学方法 甲胎蛋白 glucosidase 糖苷酶
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Antidiabetic potential of methanol extracts from leaves of Piper umbellatum L. and Persea americana Mill.
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作者 Guy Sedar Singor Njateng Sumera Zaib +6 位作者 Larissa Yetenge Chimi Cesaire Feudjio Raymond Simplice Mouokeu Donatien Gatsing Jules-Roger Kuiate Ezekiel Adewole Jamshed Iqbal 《Asian Pacific Journal of Tropical Biomedicine》 SCIE CAS 2018年第3期160-165,共6页
Objective: To determine inhibitory activity of methanolic leaf extract of Piper umbellatum and Persea americana(P. americana)(traditionally used in Cameroon against diabetes) on β-glucosidase, β-glucosidase, maltase... Objective: To determine inhibitory activity of methanolic leaf extract of Piper umbellatum and Persea americana(P. americana)(traditionally used in Cameroon against diabetes) on β-glucosidase, β-glucosidase, maltase-glucoamylase, aldose reductase and aldehyde reductase activities, enzymes involved in starch digestion or diabetic complications. Methods: The methanol extracts from Piper umbellatum and P. americana were prepared by maceration. To assess relative efficacy of these extracts, the determination of concentrations that were needed to inhibit 50% of enzyme activity was done, whereas, gas chromatography-mass spectrum was used to identify components from extracts that may be responsible for the activities. Results:The tested extracts strongly inhibited β-glucosidase, maltase-glucoamylase, aldose reductase and aldehyde reductase activities with IC50 ranging from(1.07 ± 0.03) to(31.77 ± 1.17) μg/mL.Among the tested extracts, P. americana was the most active against sensitive enzymes(IC50 of1.07 ± 0.03 to 15.63 ± 1.23). But, none of the extracts showed interesting inhibitory effect against β-glucosidase as their percentage inhibitions were less than 16%. From gas chromatographymass spectrum analysis, 10 and 8 compounds were identified in Piper umbellatum and P.americana extracts respectively, using NIST library 2014. Conclusions: Results of this study provide the scientific credential for a prospective usage of these plants to treat diabetes. 展开更多
关键词 Piper umbellatum Persea americana glucosidase Maltase-glucoamylase Aldose reductase Aldehyde reductase
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Renal hemodynamics change and renal dysfunction during liver cirrhosis
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《World Journal of Gastroenterology》 SCIE CAS CSCD 1998年第2期44-44,共1页
RenalhemodynamicschangeandrenaldysfunctionduringlivercirhosisZHANGYuanQing1,CHENYuChun4,CHENAnBing2,LIZhi... RenalhemodynamicschangeandrenaldysfunctionduringlivercirhosisZHANGYuanQing1,CHENYuChun4,CHENAnBing2,LIZhiQun3andDINGZhong... 展开更多
关键词 LIVER cirrhosis/physiopathology kidney/physiopathology hepatorenal syndrome/diagnosis HEMODYNAMICS betaglucosidase/urine
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Pompe病的研究进展 被引量:2
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作者 金梅花 金正勇 《中国妇幼保健》 CAS 北大核心 2014年第33期5541-5544,共4页
Pompe's病又称为Ⅱ型糖原累积病(glycogen storage disease,typeⅡ,GSDⅡ),由荷兰病理学家Pompe于1932年首先提出〔1〕,是一种由于先天性酸性α-葡萄糖苷(acidα-glucosidase,GAA)缺陷所导致的常染色体隐性遗传性代谢性疾病。近年... Pompe's病又称为Ⅱ型糖原累积病(glycogen storage disease,typeⅡ,GSDⅡ),由荷兰病理学家Pompe于1932年首先提出〔1〕,是一种由于先天性酸性α-葡萄糖苷(acidα-glucosidase,GAA)缺陷所导致的常染色体隐性遗传性代谢性疾病。近年来,特别是2006年美国FDA批准Myozyme作为Pompe's病的酶替代治疗(enzyme replacement therapy,ERT)药物以来,对GSDII的自然过程、Myozyme的疗效、免疫反应、病理、新生儿筛查、运动规律、陪伴治疗及基因治疗等方面取得了重要的进展,现就近年来研究进展综述如下。 展开更多
关键词 Ⅱ型糖原累积病 新生儿筛查 病理学家 代谢性疾病 glucosidase GLYCOGEN 常染色体隐性 Pompe's免疫反应 心肌病
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The effects of simulated nitrogen deposition on extracellular enzyme activities of litter and soil among different-aged stands of larch 被引量:14
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作者 Yuecun Ma Biao Zhu +3 位作者 Zhenzhong Sun Chuang Zhao Yan Yang Shilong Piao 《Journal of Plant Ecology》 SCIE 2014年第3期240-249,共10页
Aims Nitrogen(N)addition could affect the rate of forest litter and soil organic matter decomposition by regulating extracellular enzyme activity(EEa).The impact of N addition on EEa may differ across different age st... Aims Nitrogen(N)addition could affect the rate of forest litter and soil organic matter decomposition by regulating extracellular enzyme activity(EEa).The impact of N addition on EEa may differ across different age stands with different organic matter quality.We were interested in whether the impact of N addition on EEa in litter and mineral soil during the growing season was dependent on stand age of a larch plantation in North China.Methods We added three levels of N(0,20 and 50 kg N ha^(−1) year^(−1))in three age stands(11,20 and 45 years old)of Larix principis-rupprech-tii plantation in North China.We measured potential activities of β-1,4-glucosidase(b),cellobiohydrolase(Cb),β-1,4-N-acetyl-glucosaminidase(Nag)and phenol oxidase(Po)in litter(organic horizon)and mineral soil(0-10 cm)during the second growing sea-son after N amendment.We also measured C and N concentrations,microbial biomass C and N,and KCl-extractable ammonium and nitrate in both litter and mineral soil.Important Findings We observed unimodal patterns of EEa during the growing season in all three stands,consistent with the seasonal variations of soil temperature.stand age had a strong effect on EEa in both litter and mineral soil,and this effect differed between litter and mineral soil as well as between different enzymes.N addition did not significantly affect the activities of b or Cb but significantly suppressed the activity of Nag in litter.We also found stand age-specific responses of Po activity to N addition in both litter and mineral soil.N addition suppressed Po activity of the high C:N ratio litters in 20-and 45-year-old stands but had no significant effect on Po activity of the low C:N ratio litter in 11-year-old stand.moreover,N addition inhibited Po activity of the high C:N ratio soil in 20-year-old stand but had no significant impact on Po activity of the low C:N ratio soils in 11-and 45-year-old stands.overall,stand age had a greater effect on EEa in litter and mineral soil compared to 2 years of N addition.moreover,the effect of N addition on Po activity is stand age dependent,which may affect the long-term soil carbon storage in this forest. 展开更多
关键词 glucosidase CELLOBIOHYDROLASE GLUCOSAMINIDASE phenol oxidase Larix plantation
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RP-HPLC Determination of 1,3-Dideoxygalactonojirimycin in Bombycis Faeces
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作者 QI Hui,ZHAO Hui,DING Zhen-ying,ZHU Yuan-yuan Tianjin Key Laboratory of Molecular Drug Research,College of Pharmacy,Nankai University,Tianjin 300071,China 《Chinese Herbal Medicines》 CAS 2011年第2期156-158,共3页
Objective To establish a simple and rapid method for the determination of 1,3-dideoxygalactonojirimycin in Bombycis Faeces,a potent glucosidase inihibitor,by HPLC.Methods A RP-HPLC method with fluorescence detection h... Objective To establish a simple and rapid method for the determination of 1,3-dideoxygalactonojirimycin in Bombycis Faeces,a potent glucosidase inihibitor,by HPLC.Methods A RP-HPLC method with fluorescence detection has been developed.Results The HPLC method developed in this research has a good reliability including accuracy and precision.The detection limit was less than 72 ng.Conclusion This method is sufficiently sensitive for determining 1,3-dideoxygalactonojirimycin in Bombycis Faeces and other related products. 展开更多
关键词 Bombycis Faeces 1 3-dideoxygalactonojirimycin extraction glucosidase inhibitor RP-HPLC
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