排序方式: 共有6条查询结果,搜索用时 31 毫秒
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Kozyrev SV Abelson AK Wojcik J Zaghlool A Linga Reddy MV Sanchez E Gunnarsson I Svenungsson E Sturfelt G Jönsen A Truedsson L Pons-Estel BA Witte T D'Alfonso S Barizzone N Barrizzone N Danieli MG Gutierrez C Suarez A Junker P Laustrup H González-Escribano MF Martin J Abderrahim H Alarcón-Riquelme ME 《Nature genetics》2008,40(2):211-216
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Kozyrev SV Abelson AK Wojcik J Zaghlool A Linga Reddy MP Sanchez E Gunnarsson I Svenungsson E Sturfelt G Jönsen A Truedsson L Pons-Estel BA Witte T D'Alfonso S Barrizzone N Danieli MG Gutierrez C Suarez A Junker P Laustrup H Francisca González-Escribano M Martin J Abderrahim H Alarcón-Riquelme ME 《Nature genetics》2008,40(4):484
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Ramzi Chalghaf Walid Oueslati Marwa Ammar Hafsia Ben Rhaiem AbdesslemBen Haj Amara 《自然科学进展(英文版)》2013,23(1):23-35
This work aims at investigating the strain effect,created by varying pH solution and continuous heating cycle,on the cation exchange process in the case of Na-rich montmorillonite sample in contact with bi-ionic solution with variable concentration,saturated respectively by Co2+ and Cu2+ cations.The ionic exchange process is characterized using XRD analysis obtained through the comparison of experimental XRD patterns with calculated ones,which allowed us to determine several structural parameters related to the nature,abundance,size,position and organization of exchangeable cation and water molecule in the interlamellar space along the caxis.Indeed,the proposed theoretical models,for the stressed samples,show that the structure presents an interstratified hydration character and proves the coexistence of more than two ’’crystallite’’ specie in the structure.The perturbation types have an obvious effect on the selective exchange process for all stressed samples,where the interlayer space is characterized by the coexistence of more one exchangeable cation. 相似文献
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采用准静态方法测定了368.75~423.00 K温度范围内1,6-己二异氰酸酯(HDI)单组分的饱和蒸汽压,以及HDI与癸二酸二辛酯(DOS)在369.81~424.55 K温度范围内的气液相平衡数据。借助Aspen Plus软件对实验数据进行回归,得到了溶液理论中非随即(局部)双液体(NRTL)模型方程、Wilson模型参数及关联偏差,结果表明,两种模型拟合得到的温度平均绝对偏差均小于1.55 K。进而计算了二元组分相对挥发度,发现HDI与DOS在0.300~0.900 kPa范围内存在最高共沸现象,共沸物中HDI的摩尔分数为0.105~0.112。因此,在HDI精制分离时,循环溶剂中会携带10%左右的HDI。 相似文献
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Lambrechts D Storkebaum E Morimoto M Del-Favero J Desmet F Marklund SL Wyns S Thijs V Andersson J van Marion I Al-Chalabi A Bornes S Musson R Hansen V Beckman L Adolfsson R Pall HS Prats H Vermeire S Rutgeerts P Katayama S Awata T Leigh N Lang-Lazdunski L Dewerchin M Shaw C Moons L Vlietinck R Morrison KE Robberecht W Van Broeckhoven C Collen D Andersen PM Carmeliet P 《Nature genetics》2003,34(4):383-394
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Ramirez A Heimbach A Gründemann J Stiller B Hampshire D Cid LP Goebel I Mubaidin AF Wriekat AL Roeper J Al-Din A Hillmer AM Karsak M Liss B Woods CG Behrens MI Kubisch C 《Nature genetics》2006,38(10):1184-1191
Neurodegenerative disorders such as Parkinson and Alzheimer disease cause motor and cognitive dysfunction and belong to a heterogeneous group of common and disabling disorders. Although the complex molecular pathophysiology of neurodegeneration is largely unknown, major advances have been achieved by elucidating the genetic defects underlying mendelian forms of these diseases. This has led to the discovery of common pathophysiological pathways such as enhanced oxidative stress, protein misfolding and aggregation and dysfunction of the ubiquitin-proteasome system. Here, we describe loss-of-function mutations in a previously uncharacterized, predominantly neuronal P-type ATPase gene, ATP13A2, underlying an autosomal recessive form of early-onset parkinsonism with pyramidal degeneration and dementia (PARK9, Kufor-Rakeb syndrome). Whereas the wild-type protein was located in the lysosome of transiently transfected cells, the unstable truncated mutants were retained in the endoplasmic reticulum and degraded by the proteasome. Our findings link a class of proteins with unknown function and substrate specificity to the protein networks implicated in neurodegeneration and parkinsonism. 相似文献
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