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Spectroscopy of Low-lying States in Odd-Z Odd-A Nuclei Beyond Lead

原子核物理评论 2016年第2期33卷 141-145页

作者： U.jakobsson j.Uusitalo K.Auranen H.Badran B.Cederwall D.M.Cox T.Grahn P.T.Greenlees R.julin S.juutinen A.Herzán j.Konki M.Leino M.Mallaburn j.Pakarinen P.Papadakis j.partanen P.Rahkila M.Sandzelius j.Sarén C.Scholey j.Sorri S.Stolze KTH The Division of Nuclear PhysicsAlba Nova University Center University of jyvaskyla Department of Physics

Low-lying states in odd-Z odd-mass nuclei at the proton drip-line beyond lead have recently been studied through fusion-evaporation reactions using a gas-filled recoil separator. Isomeric 1/2+and13/2+states have been observed in odd-mass astatine and francium nuclei. The systematic behaviour of the level energies of these states have been studied and a similarity between the 1/2+state in astatine and francium has been found. Furthermore, the 13/2+state has been observed in the francium nuclei with an oblate behaviour suggesting a coupling of the i13/2proton to the 2p- 2h intruder excitation.

关键词： Nuclear spectroscopy isomeric states Z > 82

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组织蛋白酶D缺乏导致人类先天性神经元蜡样脂褐质沉着症

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世界核心医学期刊文摘(神经病学分册) 2006年第11期 7-8页

作者： Siintola E partanen S Strmme P j. Tyynel 周永 Institute of Biomedicine/Biochemistry University of Helsinki Haartmaninkatu 8 00014 Helsinki Finland Dr.

Congenital neuronal ceroid-lipofuscinosis (NCL) is a devastating inherited neurodegenerative disorder of unknown metabolic basis. Eight patients with this rare disorder, all with similar clinical and neuropathological findings, have been reported, and here we describe two further patients. Previously, we showed that a mutation in the cathepsin D gene causes congenital NCL in sheep. On the basis of the neuropathological and ultrastructural similarities between the sheep and patients affected with congenital NCL, we screened the cathepsin D gene for mutations in a patient of Pakistani origin. We identified a nucleotide duplication, c.764dupA, in the cathepsin D gene in homozygous form in the patient, and in heterozygous form in his father. This duplication is likely to be disease causing, as it creates a premature stop codon, predicting a truncation of the protein. When transiently expressed in cell cultures, the mutant protein was enzymatically inactive, but stable. In paraffin-embedded brain tissue samples of two affected siblings of the Pakistani patient, cathepsin D was absent, suggesting rapid degradation of the c.764dupA mutant cathepsin D at mRNA or protein level in vivo. Further, we were able to confirm lack of cathepsin D in the brain tissue of yet another, unrelated, patient of English origin with congenital NCL. On the basis of the present data, and the nearly identical clinical and/or pathological phenotype of the other reported cases of congenital NCL, it is reasonable to suggest that cathepsin D deficiency caused by mutations in the corresponding gene may underlie all cases of congenital NCL. The present observations also suggest that cathepsin D deficiency should be considered as a possible diagnosis in microcephalic neonates, who present with seizures at or before birth.

关键词：组织蛋白酶基因突变脂褐质点突变蜡样神经组织神经元

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Radiation detectors fabricated on high-purity GaAs epitaxial materials

Radiation detectors fabricated on high-purity GaAs epitaxial...

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第十三届全国物理力学学术会议

作者： X.P.Wu M.Mattila T.Riekkinen A.Gdda M.partanen P.Kostamo S.Nenonen H.Lipsanen j.Salonen H.Pohjonen S.Er?nen A.Oja VTT Aalto University Oxford Instruments Analytical Oy Tarvonsalmenkatu 17EspooFinland

A CVPE(Chloride Vapor Phase Epitaxy)reactor was built to grow high purity epitaxial Ga As *** reactor is made of high purtity quartz and designed to minimize air *** epitaxial layer is grown by introducing high purtiy As Cl3and purified H2 gas to a two-zone furnace where the Ga melt and the seed crystals are positioned in sequence and at appropriate *** reactor has verticl

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