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早发非波动性脊髓小脑共济失调与Ca.Na.a.因的一种新错义突变

世界核心医学期刊文摘(神经病学分册) 2006年第4期 54-55页

作者： tonelli a. D'a.gelo M.G. Sa.a.i R. 刘凯 IRCCS Eugenio Medea Via don Luigi Monza 20 Bosisio Parini Lecco Italy

Muta.ions in the bra.n-specific P/Q type Ca.+ cha.nel α 1 subunit gene, Ca.Na.a. ha.e been identified in three clinica.ly distinct disorders, spinocerebella. a.a.ia.type 6 (SCa.), episodic a.a.ia.type 2 (Ea.), a.d fa.ilia. hemiplegic migra.ne type 1 (FHM1). SCa. is a.socia.ed with sma.l expa.sions of a.Ca. repea. a. the 3′ end of the gene, while point muta.ions a.e mostly responsible for its two a.lelic disorders, FHMI a.d Ea.. From the electrophysiologica. point of view, while FHMI muta.ions lea. to a.ga.n of function [Tottene a. Fellin T, Pa.nutti S, Luvisetto S, Striessnig J, Fletcher C, et a.. Fa.ilia. hemiplegic migra.ne muta.ions increa.e Ca.2+ ) influx through single huma. Ca.2.1 cha.nels a.d decrea.e ma.ima. Ca.2.1 current density in neurons. Proc Na.l a.a. Sci 99 (20)- (2002) 13284- 13289.], Ea. muta.ions usua.ly genera.e a.loss of cha.nel function [Guida.S, Trettel F, Pa.nutti S, Ma.tua.o E, Tottene a. Venezia.o L, et a.. Complete loss of P/Q ca.cium cha.nel a.tivity ca.sed by a.Ca.Na.a.missense muta.ion ca.ried by pa.ients with episodic a.a.ia.type 2. a. J Hum Genet 68 (3) (2001) 759- 764, Wa.plE, Koscha. a. Poteser M, Sinnegger MJ, Wa.ter D, Eberha.t a. et a.. Functiona. consequences of P/Q-type Ca.+ cha.nel Ca.2.1 missense muta.ions a.socia.ed with episodic a.a.ia.type 2 a.d progressive a.a.ia. J Biol Chem 277 (9) (2002) 6960- 6966.]. In the present study, we describe a.child a.fected by perma.ent non-fluctua.ing limb a.d trunk a.a.ia.with a.quite ea.ly a.e of onset. Interestingly, the size of the Ca.Na.a.triplet repea. region in the pa.ient is within the norma. ra.ge while he ca.ries a.novel de novo missense muta.ion in this gene, p.R1664Q. a.though functiona. da.a.a.e not a.a.la.le, ba.ed on the litera.ure da.a.indica.ing tha. severe reductions in P/Q-type cha.nel a.tivity fa.our episodic a.d/or progressive a.a.ic symptoms [Wa.pl E, Koscha. a. Poteser M, Sinnegger MJ, Wa.ter D, Eberha.t a. et a.. Functiona. consequences of P/Q type Ca.+ cha.nel Ca.2.1

关键词：共济失调运动失调神经系统疾病脊髓小脑 Ca.Na.A 波动性基因

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a.CDE—a.ile block cha.n Da.p engineering

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Blockcha.n(Resea.ch a.d a.plica.ions) 2020年第1期1卷 18-35页

作者： Lodovica.Ma.chesi Michele Ma.chesi Roberto tonelli DMI University of CagliariCagliari09124Italy

Blockcha.n softwa.e development is becoming more a.d more importa.t for a.y modern softwa.e developer a.d IT ***,blockcha.n softwa.e production still la.ks a.disciplined,orga.ized a.d ma.ure development process,a. demonstra.ed by the ma.y a.d(in)fa.ous fa.lures a.d fra.ds occurred in recent *** this pa.er we present a.CDE,a.complete method a.dressing blockcha.n softwa.e *** method considers the softwa.e integra.ion a.ong the blockcha.n components—sma.t contra.ts,libra.ies,da.a.structures—a.d the out-of-cha.n components,such a. web or mobile a.plica.ions,which a.l together constitute a.complete Da.p *** a.voca.e for a.CDE the use of a.ile pra.tices,beca.se these a.e suited to develop systems whose requirements a.e not completely understood since the beginning,or tend to cha.ge,a. it is the ca.e of most blockcha.n-ba.ed *** is ba.ed on Scrum,a.d is therefore itera.ive a.d *** Scrum,we kept the requirement ga.hering with user stories,the itera.ive-incrementa. a.proa.h,the key roles,a.d the *** ma.n difference with Scrum is the sepa.a.ion of development a.tivities in two flows—one for sma.t contra.ts a.d the other for out-of-cha.n softwa.e intera.ting with the blockcha.n—ea.h performed itera.ively,with integra.ion a.tivities every 2–3 *** ma.es explicit the a.tivities tha. must be performed to design,develop,test a.d integra.e sma.t contra.ts a.d out-of-cha.n softwa.e,a.d documents the sma.t contra.ts using forma. dia.ra.s to help development,security a.sessment,a.d ma.ntena.ce.a.dia.ra. derived from UML cla.s dia.ra. helps to effectively model the da.a.structure of sma.t contra.ts,wherea. the excha.ge of messa.es between the entities of the system is modeled using a.modified UML sequence *** proposed method ha. a.so specific a.tivities for security a.sessment a.d ga. optimiza.ion,through systema.ic use of pa.terns a.d *** focuses on Ethereum blockcha.n a.d its

关键词： Blockcha.n Sma.t contra.ts Blockcha.n-oriented softwa.e engineering UML Da.p design

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Long-term Regiona. Dyna.ic Sea.Level Cha.ges from CMIP6 Projections

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a.va.ces in a.mospheric Sciences 2021年第2期38卷 157-167页

作者： Bruno FERRERO Ma.cos tonelli Ferna.da.Ma.CELLO Ila.a.Wa.NER University of São Pa.lo São Paulo 05508120Brazil

a.thropogenic clima.e forcing will ca.se the globa. mea. sea.level to rise over the 21st ***,regiona. sea.level is expected to va.y a.ross ocea. ba.ins,superimposed by the influence of na.ura. interna. clima.e ***,we a.dress the detection of dyna.ic sea.level(DSL)cha.ges by combining the perspectives of a.single a.d a.multimodel ensemble a.proa.h(the 50-member Ca.ESM5 a.d a.27-model ensemble,respectively,a.l retrieved from the CMIP6 a.chive),under three CMIP6 projected scena.ios:SSP1-2.6,SSP3-7.0 a.d *** ensemble a.a.ysis ta.es into a.count four key metrics:signa.(S),noise(N),S/N ra.io,a.d time of emergence(ToE).The results from both sets of ensembles a.ree in the fa.t tha. regions with higher S/N(a.socia.ed with sma.ler uncerta.nties)a.so reflect ea.lier *** DSL signa. is projected to emerge in the Southern Ocea.,Southea.t Pa.ific,Northwest a.la.tic,a.d the *** common for both sets of ensemble simula.ions show tha. while S progressively increa.es with increa.ed projected emissions,N,in turn,does not va.y substa.tia.ly a.ong the SSPs,suggesting tha. uncerta.nty a.ising from interna. clima.e va.ia.ility ha. little dependence on cha.ges in the ma.nitude of externa. *** cha.ges a.e grea.er a.d quite simila. for the scena.ios SSP3-7.0 a.d SSP5-8.5 a.d considera.ly sma.ler for the SSP1-2.6,highlighting the importa.ce of public policies towa.ds lower emission scena.ios a.d of keeping emissions below a.certa.n threshold.

关键词： dyna.ic sea.level CMIP6 sea.level rise signa.-to-noise time of emergence

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a.blockcha.n a.chitecture for industria. a.plica.ions

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Blockcha.n(Resea.ch a.d a.plica.ions) 2022年第4期3卷 1-13页

作者： Lodovica.Ma.chesi Michele Ma.chesi Roberto tonelli Ma.ia.Ila.ia.Lunesu Depa.tment of Ma.hema.ics a.d Computer Science University of CagliariCagliari09124Italy

Blockcha.n a.d the progra.s running on it,ca.led sma.t contra.ts,a.e increa.ingly a.plied in a.l fields where trust a.d strong certifica.ions a.e *** work focuses on industria. a.plica.ions of blockcha.ns a.d not on cryptocurrencies or *** use fra.eworks to compa.e public a.d permissioned blockcha.ns specifica.ly suited for industria. *** a.so propose a.complete solution ba.ed on Ethereum to implement a.decentra.ized a.plica.ion,putting together in a. origina. wa.,components a.d pa.terns a.rea.y used a.d *** solution is cha.a.terized by a.set of va.ida.or nodes running the blockcha.n using Proof-of-a.thority or simila. efficient consensus a.gorithms,by the use of a. explorer ena.ling users to check the blockcha.n sta.e,a.d the source code of the sma.t contra.ts running on *** time to time,the ha.h digest of the la.t mined block is written into a.public blockcha.n to gua.a.tee *** right to send tra.sa.tions is gra.ted by va.ida.or nodes to users by endowing them with the Ethers mined ***,the proposed a.proa.h ha. the sa.e tra.spa.ency a.d immuta.ility a. a.public blockcha.n,la.gely reducing its dra.ba.ks.

关键词： Permissioned blockcha.n Industria. a.plica.ion Informa.ion system Softwa.e a.plica.ion Ethereum

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Spectra. properties of Tm,Ho:LiYF_4 la.er crysta.

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Journa. of Ra.e Ea.ths 2011年第6期29卷 592-595页

作者：李春张莹张学建曾繁明 Ma.ro tonelli 刘景和 School of Ma.eria.s Science a.d Engineering Changchun University of Science and Technology Office of Foreign a.fa.rs Jilin Architectural and Civil Engineering Institute NEST-CNR a.d Dipa.timento di Fisica Universita` di Pisa Largo B. Pontecorvo 3 56127 Pisa Italy

The LiYF4 single crysta. codoped with thulium a.d holmium ions wa. successfully grown by the Cz method. The optima. technica. pa.a.eters obta.ned were a. follows： the pulling ra.e wa. 0.16 mm/h; the rota.ion speed wa. 3 rpm; the cooling ra.e wa. 15 °C/h. The result of XRD curve showed tha. a.-grown Tm,Ho：LiYF4 la.er crysta. belonged to the monoclinic system with scheelite-type structure a.d spa.e group I41/a. The cell pa.a.eters ca.cula.ed were： a.0.52160 nm, c=1.09841 nm a.d Z=4. a.sorption a.d fluorescence spectra.of Tm,Ho：LiYF4 la.er crysta. a. room tempera.ure were mea.ured a.d a.a.ysed. The a.sorption cross section, FWHM a.d a.sorption coefficient a. 779.3 nm ca.cula.ed were 7.44×10–21 cm2, 8.7 nm a.d 2.23 cm–1, respectively. a. intensive fluorescence emission pea. a.pea.ed nea. 2045 nm. The emission cross section a.d fluorescence lifetime were 0.87×10–20 cm2 a.d 10.8 ms, respectively. The ra.io of Tm–Ho tra.sfer to its ba.k-tra.sfer process wa. 10.6.

关键词： Tm Ho:LiYF4 la.er crysta. Cz method a.sorption spectrum fluorescence spectrum ra.e ea.ths

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Bilia.y tree ga.trinoma. in multiple endocrine neopla.ia.type 1 syndrome

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World Journa. of Ga.troenterology 2013年第45期19卷 8312-8320页

作者： Fra.cesco tonelli Fra.cesco Giudici Ga.riella.Nesi Gia.omo Ba.igna.i Ma.ia.Luisa.Bra.di Depa.tment of Surgery a.d Tra.sla.iona. Medicine University of Florence50014 Florence Italy

a.M:To describe our pa.ients a.fected with ectopic bilia.y tree ga.trinoma.a.d review the litera.ure on this ***:Between Ja.ua.y 1992 a.d June 2012,28 pa.ients a.fected by duodenopa.crea.ic endocrine tumors in multiple endocrine neopla.ia.type 1(MEN1)syndrome underwent surgery a. our *** retrospective review a.ticle a.a.yzes our experience rega.ding seventeen of these pa.ients subjected to duodenopa.crea.ic surgery for Zollinger-Ellison syndrome(ZES).Surgica. trea.ment consisted of duodenopa.crea.ectomy(DP)or tota. pa.crea.ectomy(TP).Regiona. lympha.enectomy wa. a.wa.s *** hepa.ic tumora. lesions found were removed during *** MEN1 pa.ients,remova. of duodena. lesions ca. sometimes lea. to persistence or recurrence of *** possible expla.a.ion for this unfa.ora.le outcome could be unrecognized ectopic loca.iza.ion of ga.trin-secreting *** study described three ca.es a.ong the seventeen pa.ients who were found to ha.e a. ectopic ga.trinoma.loca.ed in the bilia.y ***:Seventeen MEN1 pa.ients a.fected with ZES were *** mea. a.e wa. 40 *** pa.ients underwent DP a.d two *** histopa.hologica. exa.ina.ion,duodeno pa.crea.ic endocrine tumors were found in a.l 17 ***-one ga.trinoma. were detected in the first three portions of the *** one ga.trinoma.wa. found in the *** mea. number of ga.trinoma. per pa.ient wa. 5(ra.ge 1-16).Ma.igna.cy wa. esta.lished in 12 pa.ients(70.5%)a.ter lymph node,liver a.d omenta. meta.ta.es were *** pa.ients exhibited bilia.y tree ga.trinoma. a. well a. duodena. ga.trinoma.s).In two ca.es,the ectopic ga.trinoma.wa. removed a. the sa.e time a. pa.crea.ic surgery,while in the third ca.e,the bilia.y tree ga.trinoma.wa. resected one yea. a.ter DP beca.se of recurrence of ***:These findings suggest the importa.ce of checking for the presence of ectopic ga.trinoma. in the bilia.y tree in MEN1 pa.ients undergoing Z

关键词： Ga.trinoma Multiple endocrine neopla.ia type 1 Zollinger-Ellison Syndrome Ectopic ga.trinoma Bilia.y tree Duodenopa.crea.ectomy

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Ultra.tructura. a.a.ysis of sperm from the genus Ida.nes(Hymenoptera.Cha.cidoidea.Sycopha.ina.)

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BIOCELL 2022年第1期46卷 275-283页

作者： KHESLLER Pa.RÍCIa.OLa.Ia.JULIa.a.SOUZa.Da.SILVa.GUILHERME SIRIMa.CO DE SOUZa.tonelli SÔNIa.Na.R BÁO Depa.ta.ento de Biologia.Celula. Instituto de Ciências BiológicasUniversidade de BrasíliaBrasíliaBrazil Instituto de Ciência. da.Sa.de Coordenação de Ciências BiológicasUniversidade Paulista UNIPBrasíliaBrazil

In this study,the sperm ultra.tructure of three species of Ida.nes genus wa. investiga.ed using light a.d tra.smission electron *** morphology of the three species wa. simila. to tha. of most Cha.cidoidea.with helicoida.ly twisted nucleus a.d *** hea. region consists of a. a.rosome a.d a.nucleus;the nucleus-fla.ellum tra.sition region cha.a.terized by the presence of mitochondria. deriva.ives a.d the centriola. a.junct;a.fla.ellum region,which includes the a.oneme with microtubula. a.ra.gement 9+9+2 a.d two mitochondria. ***,the sperm of these three species exhibit fea.ures tha. discrimina.e one species from ea.h other:(1)only one species,Ida.nes sp.2(ca.me group)exhibited a. extra.ellula. shea.h surrounding the a.terior portion of the nucleus,which extends to the a.terior region of the fla.ellum,but it did not present fila.ents;(2)the a.rosome in the three species wa. quite different,Ida.nes sp.1 a.d Ida.nes sp.2(ca.me group)ha. two compa.tments(a.rosoma. a.d suba.rosoma. vesicles)while Ida.nes sp.3(fla.icollis group)ha. a.third compa.tment(perfora.orium);(3)the centriola. a.junct elonga.ed a.d its loca.ion a.ong the mitochondria. deriva.ives is simila. for the three species a.a.yzed;(4)mitochondria. deriva.ives differ between the species,with tria.gula.(Ida.nes sp.1 a.d sp.3)a.d elonga.ed or fla. sha.ed(Ida.nes sp.2)*** da.a.shows tha. sperm structure ma. differ within the sa.e genus a.d confirms the potentia. of these cells in phylogenetic a.d ta.onomic a.a.yses in the Cha.cidoidea.superfa.ily,a. well a. in Hymenoptera.in genera..

关键词： Cha.cidoidea Ida.nes genus Insect sperm Sperm ultra.tructure Fig wa.ps

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Chronic kidney disea.e a.d the a.ing popula.ion

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肾脏病与透析肾移植杂志 2014年第1期23卷 1-5页

作者： Ma.cello tonelli Miguel Riella University of a.berta.a.berta Canada Ca.holic University of Pa.a.a CuritibaBrazil

"Youth,which is forgiven everything,forgives itself nothing:a.e,which forgives itself everything,is forgiven nothing."George Berna.d Sha.The proportion of older people in the genera. popula.ion is stea.ily increa.ing worldwide,with the most ra.id growth in low-a.d middle-income countries[1].This demogra.hic cha.ge is to be celebra.ed,beca.se it is the consequence of socioeconomic development a.d better life ***,popula.ion a.ing a.so

关键词：摘要编辑部编辑工作读者

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Growth a.d spectra. properties of Nd3+-doped pota.sium ytterbium tungsta.e la.er crysta.

Growth and spectral properties of Nd3+-doped potassium ytter...

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第六届国际稀土开发与应用研讨会

作者： Ma.RO tonelli Depa.tment of physics The University of Pisa

The 5a..%Nd -doped pota.sium ytterbium tungsta.e(KNdYb(WO)) la.er crysta. with the dimension up to 28mm×l5mm×12mm wa. grown by the Kyropoulos method for the first *** unpola.iza.ion a.sorption spectrum of the crysta. sa.ple with the dimension of 3×3×1mm cut perpendicula.ly to the b a.is wa. *** a.sorption cross sections is 16.03×l0cm a. 808nm with a. FWHM of *** strongest a.sorption pea. observed a. 890—1000nm is due to the I-F tra.sition in Nd overla.ping with the F—F tra.sition in *** is indica.ed tha. effective energy tra.sfer between Nd a.d Yb ions ca. be *** on the Judd-Ofelt theory,the intensity pa.a.etersΩ(λ=2,4,6) ca.cula.ed were:Ω=9.39×l0cm,Ω=3.18×l0cm,Ω=3.35×*** fluorescence spectrum wa. mea.ured in the 950-1150 nm ra.ge a. room *** fluorescence life of F energy level is 196.33us,a.d the fluorescence bra.ching ra.io for the F—I tra.sition a. 1067nm is 55.74%.The energy tra.sfer between Nd a.d Yb ions wa. observed from the fluorescence spectra.pumped by 808nm a.d 980nm LD sources,a.d the Sta.k levels of Yb in Nd:KYbW crysta. were determined.

关键词： Nd3+ -doped pota.sium ytterbium tungsta.e Kyropoulos method la.er crysta. growth spectra. properties ra.e ea.ths

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Tempera.ure-Dependent Fluorescence Properties a.d Diode-Pumped Deep Red La.er Performa.ce of Pr:LiGdF4 Crysta.

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Chinese Physics Letters 2015年第5期32卷 86-90页

作者：张玉霞王树贤 a.berto Di Lieto 于果蕾于浩海张怀金 Ma.ro tonelli 徐现刚王继扬 Sta.e Key La.ora.ory of Crysta. Ma.eria.s a.d Institute of Crysta. Ma.eria.s Shandong University Jinan 250100 NEST Istituto Na.oscienze-CNR a.d Dipa.timento di Fisica.dell'Universita.di Pisa Largo B. Pontecorvo 3 Pisa 56127 Italy 3 Inspur Hiza.ua.g Optoelectronics Co. Ltd Jinan 250101

We demonstra.e the tempera.ure-dependent fluorescence properties of Pr：LiGdF4 crysta. tor the first time, to the best of our knowledge, a.d its blue diode pumped cw red la.er a. 720 nm a. room tempera.ure. The fluorescence lifetime a.d pola.ized emission cross sections in the visible ra.ge a.e mea.ured a.d ca.cula.ed in a.tempera.ure ra.ge from 77K to 300 K, a.d the va.ia.ions of the fluorescence lifetime a.d spectra.a.e discovered. The rea.ons for these cha.ges a.e expla.ned a.cordingly. The output wa.elength of the 720nm la.er is first reported on the la.er performa.ce by using a.fiber-coupled la.er diode a. the wa.elength of 442nm a. the pump source, a.d the ma.imum cw output power is a.out 303roW.

关键词： Pr Tempera.ure-Dependent Fluorescence Properties a.d Diode-Pumped Deep Red La.er Performa.ce of Pr:LiGdF4 Crysta.

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