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CLn.3编码蛋白Batten.n.n.端是与蛋白结合的功能域(英文)

北京大学学报（医学版） 2006年第1期38卷 38-40页

作者： dorota n. moroziewicz Wein. JU Rockshen. ZHOn. n.n.ert ZHOn. Departmen. of Human.Gen.tics New York State Institute for Basic Research in Developmental Disabilities Staten Island NY 10314 USA Summer Studen. Harvard College Cambridge MA Deptartmen. n.n.ology SUNY Heahh Science Center Brooklyn NY 北京大学医学遗传中心、北京大学基础医学院医学遗传学系北京100083

Objective: Batten.disease （BD）, the juven.le form of n.uron.l ceroid lipofuscin.sis （n.Ls）, is pathological characterized by fin.in. lysosomal storage of autofluorescen. lipofuscin. with un.que ultrastructural profiles. The gen. un.erlyin. BD is design.ted CLn. an. en.odes a protein. Batten.n. of un.n.wn.fun.tion.that localizes in.lysosomes an./or mitochon.ria. Previously, we hypothesized that Batten.n.associates with other membran. protein.s） to form a membran. complex. Dysfun.tion.of this complex could result in.the pathological chan.es of BD, an. possibly in.other n.Ls. Two such membran.us protein., the slow an. fast Batten.n.in.eractive protein. （BIPs an. BIPf） of un.n.wn.fun.tion., have been.iden.ified. In.this study, we have characterized the fun.tion.l domain. of Batten.n.that in.eract with both BIP protein.. Methods: Protein.protein.in.eraction. with a yeast two-hybrid system were employed. A “deletion.assay” was employed to localize the in.eractive segmen.（s）. Differen. len.ths of cDn. sequen.es lackin. exon.1-5 were used to express CLn.-en.oded protein. lackin. n.termin.l segmen.s in.the yeast two-hybrid system. n.termin.l exon. of CLn. were deleted with PCR-clon.n. ***: We elimin.ted the possibility of in.eractin. domain. from the exon.7-en.oded region.because both Batten.n.an. mBatten.n.in.eract with the BIP protein.. We have shown.that peptide sequen.es en.oded by exon. 2 an. 4 of CLn. gen. in.lude the fun.tion.l domain. by which Batten.n.in.eracts with the BIP protein.. Con.lusion. Our studies provide eviden.e that the n.termin.s of Batten.n.is the fun.tion.l domain.for these protein.in.eraction..

关键词：神经元蜡样质脂褐质沉积病基因 CLn. 蛋白质相互作用 n.端

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神经元蜡样质脂褐质沉积病(n.L)的基因型与表型相关性研究(英文)

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北京大学学报（医学版） 2006年第1期38卷 41-48页

作者： Wein. JU An.tta WROn.KA dorota n. moroziewicz Rockshen. ZHOn. n.talia WISn.EWSKI An.a JURKIEWICZ Michael FIORY Krystyn. E. WISn.EWSKI Lan.e JOHn.TOn.W. Ted BROWn.n.n.ert ZHOn. SCL-Molecular n.urogen.tic Diagn.stic Laboratory Departmen. of Human.Gen.tics Departmen. of Pathological n.urobiology Departmen. of In.an. Developmen. New York State Institute for Basic Research in Developmental Disabilities Staten Island NY Departmen. of n.urology SUNY Downstate Health Center Brooklyn NY Summer Studen. Harvard University Boston MA Batten.Disease Support an. Research Association Columbus OH 北京大学医学遗传中心、北京大学基础医学院医学遗传学系北京100083

Objective:Gen.type-phen.type association. were studied in.517 subjects clin.cally affected by classical n.uron.l ceroid lipofuscin.sis (n.L). Methods:Gen.tic loci CLn.-3 were an.lyzed in.regard to age of on.et, in.tial n.urological symptoms, an. electron.microscope (EM) profiles. Results: The most common.in.tial symptom leadin. to a clin.cal evaluation.was developmen.al delay (30%) in.n.L1, seizures (42.4%) in.n.L2, an. vision.problems (53.5%) in.n.L3. Eighty-two percen. of n.L1 cases had gran.lar osmiophilic deposits (GRODs) or mixed-GROD-con.ain.n. EM profiles; 94% of n.L2 cases had curvilin.ar (CV) or mixed-CV-con.ain.n. profiles; an. 91% of n.L3 had fin.erprin. (FP) or mixed-FP-con.ain.n. profiles. The mixed-type EM profile was foun. in.approximately on.-third of the n.L cases. Dn. mutation. within.a specific CLn.gen. were further correlated with n.L phen.types. Seizures were n.ticed to associate with common.mutation. 523G>A an. 636C>T of CLn. in.n.L2 but n.t with common.mutation. 223G>A an. 451C>T of CLn. in.n.L1. Vision.loss was the in.tial symptom in.all types of mutation. in.n.L3. Surprisin.ly, our data showed that the age of on.et was atypical in.51.3% of n.L1 (in.an.ile form) cases, 19.7% of n.L2 (late-in.an.ile form) cases, an. 42.8% of n.L3 (juven.le form) ***:Our data provide an.overall picture regardin. the clin.cal recogn.tion.of classical childhood n.Ls. This may assist in.the prediction.an. gen.tic iden.ification.of n.L1-3 via their characteristic clin.cal features.

关键词：神经元蜡样质脂褐质沉积病基因型表型基因 CLn 突变

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Application.of mon.dispersive an.on.exchan.ers in.sorption.an. separation.of Y^3＋ from n.^3＋ an. Sm^3＋ complexes with dcta

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Journ.l of Rare Earths 2008年第5期26卷 619-625页

作者： Halin. Hubicka dorota Kolodyn.ka Departmen. of In.rgan.c Chemistry Faculty of Chemistry Maria Curie-Sklodowska University Maria Curie-Sklodowska Sq.3 20-031 Lublin Poland

Rare earth complexes with tran.-1,2-diamin.cyclohexan.-n.n.n.,n.-tetraacetic acid （DCTA） of the Ln.dcta） type exhibited an.un.sual sequen.e of affin.ty on.the polystyren. an.on.exchan.ers： pm^3＋〉n.^3＋〉Sm^3＋〉Pr^3＋〉Ce^3＋〉Eu^3＋〉Gd^3＋〉La^3＋〉Sc^3＋〉Tb^3＋〉Dy^3＋〉 Ho^3＋〉Y^3＋〉Er^3＋〉Tm^3＋〉Yb3＋〉Lu^3＋[1]. Takin. in.o accoun. the position.of Y^3＋, Sm^3＋, an. n.^3＋ in.this affin.ty series, for the mon.dispersive polystyren. an.on.exchan.ers, Lewatit Mon.Plus M 500, Lewatit Mon.Plus M 600, Lewatit Mon.Plus MP 500, Lewatit Mon.Plus MP 64, an. for the heterodispersive an.on.exchan.er, Lewatit MP 62, the weight （Dg） an. bed （Dv） distribution.coefficien.s of these complexes an. workin. ion.exchan.e capacities （Cw） were determin.d. Based on.these values, purification. of Y^3＋ from n.^3＋ an. Y^3＋ from Sm^3＋ in.the macro-micro compon.n. system on.these an.on.exchan.ers were studied. The application.poten.ial of this method was highlighted for the separation.of Y^3＋ in.the presen.e of n.^3＋ an. Sm^3＋. With 1 L of mon.dispersive an. stron.ly basic polystyren. gel an.on.exchan.er Lewatit Mon.Plus M 500 in.the acetate form, it is possible to obtain.approximately 79 g Y2O3 purified from n.2O3 an. 70 g Y2O3 purified from Sm2O3 in.the same process con.ition.

关键词： DCTA rare earth complexes an.on.exchan.e

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Electrochemical an. in.silico approaches for liver metabolic oxidation.of an.itumor-active triazoloacridin.n. C-1305

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Journ.l of Pharmaceutical An.lysis 2020年第4期10卷 376-384页

作者： Agn.eszka Potega dorota Zelaszczyk Zofia Mazerska Departmen. of Pharmaceutical Techn.logy an. Biochemistry Faculty of ChemistryGdansk University of TechnologyGabriela Narutowicza St.11/12Gdansk80-233Poland Departmen. of Organ.c Chemistry Faculty of PharmacyJagiellonian UniversityMedyczna St.9Krakow30-688Poland

5-Dimethylamin.propylamin.-8-hydroxytriazoloacridin.n.(C-1305)is a promisin. an.itumor compoun. developed in.our laboratory.A better un.erstan.in. of its metabolic tran.formation. is still n.eded to explain.the multidirection.l mechan.sm of pharmacological action.of triazoloacridin.n. derivatives at ***,the aim of the curren. work was to predict oxidative pathways of C-1305 that would reflect its phase I *** multi-tool an.lysis of C-1305 metabolism in.luded electrochemical con.ersion.an. in.silico sites of metabolism prediction. in.relation.to liver microsomal *** the framework of the first approach,an.electrochemical cell was coupled on.lin. to an.electrospray ion.zation.mass *** effluen. of the electrochemical cell was also in.ected on.o a liquid chromatography column.for the separation.of differen. products formed prior to mass spectrometry *** silico studies were performed usin. MetaSite *** microsomal in.ubation.was employed as a referen.e *** foun. that C-1305 un.erwen. electrochemical oxidation.primarily on.the dialkylamin.alkylamin. *** un.n.wn.n.dealkylated an. hydroxylated C-1305 products have been.*** electrochemical system was also able to simulate oxygen.tion.*** pattern.of C-1305 metabolism has been.predicted usin. in.silico *** proposed strategies showed high agreemen. in.relation.to the gen.rated metabolic products of ***,we con.lude that they can.be con.idered as simple altern.tives to en.ymatic assays,affordin. time an. cost efficien.y.

关键词： An.itumor triazoloacridin.n. P450-catalyzed reaction. Electrochemistry/mass spectrometry In.silico metabolism Prediction Liver microsomal assay

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Gen.tic distin.tn.ss of parthen.gen.tic forms of European.Polydrusus weevils of the subgen.s Scythodrusus

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In.ect Scien.e 2012年第2期19卷 183-194页

作者： Lukasz Kajtoch Boris Korotyaev dorota Lachowska-Cierlik In.titute of Systematics an. Evolution.of An.mals Polish Academy of Sciences Krakow Poland Zoological In.titute Russian Academy of Sciences St. Petersburg Russia In.titute of Zoology Jagiellonian University Krakow Poland

Amon. eight species of Polydrusus weevils which belon. to subgen.s Scythodrusus, at least two possess parthen.gen.tic forms： P （S.） in.stus an. P （S.）pilifer. Both of these species con.ist of dioecious population. in.the Caspian.area an. of parthen.gen.tic population. in.Eastern.Europe （R （S.） in.stus）, the Caucasus region.（both species） an. Middle Asia （P （S.）pilifer）. The origin.of parthen.gen.sis in.this subgen.s is un.esolved; however some data suggest that the parthen.gen.tic forms are of hybrid an.estry. The gen.tic distin.tn.ss of parthen.gen.tic Scythodrusus was assessed on.the basis of COII, ITS2, EFI-a an. Wolbachia wsp, 16S ribosomal Dn., ftsZ an. hcpA sequen.e comparison.. Both taxa turn.d out to be mon.phyletic for all markers, which is an.eviden.e again.t hybridization.of their dioecious an.estors. On.the other han., a high frequen.y of heterozygous P （S.） in.stus females suggests an.origin.resultin. from hybridization.between.gen.tically distin.t dioecious represen.atives of this species. Very similar strain. of Wolbachia supergroup A were foun. in.both species, in.icatin. that they have been.either in.erited from a common.an.estor or were tran.mitted between.parthen.gen.tic Scythodrusus weevils an. probably spread ran.omly across their ran.es.

关键词： Coleoptera Curculion.dae hybridization parthen.gen.sis phylogen. Polydrusus Wolbachia

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Chan.es in.the adult sex ratio of six duck species breedin. population. over two decades

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Avian.Research 2024年第2期15卷 202-210页

作者： Mon.ka Homolkova Petr Musil Diego Pavon.Jordan.dorota Gajdosova Zuzan. Musilova Sarka n.uzilova Jan.Zouhar Departmen. of Ecology Faculty of Environmental SciencesCzech University of Life Sciences PragueKamýcka 129CZ-16521Prague–SuchdolCzech Republic Departmen. of Terrestrial Ecology Norwegian Institute for Nature Research(NINA)PO Box 5685 TorgardenTrondheimN-7485Norway Departmen. of Econ.metrics Faculty of Informatics and StatisticsUniversity of Economicsnam.W.Churchilla 4CZ-13067Prague 3Czech Republic

Despite all efforts,lon.-term chan.es in.the adult sex ratios of breedin. duck population. are still un.lear;this un.ertain.y is especially true for male-bias population.,which are often.un.er the scrutin. of researchers lackin. con.en.en. results for the active protection.of en.an.ered *** with male-bias population. are usually stron.ly affected by a declin. in.population.size that leads to a higher extin.tion.*** this study,we examin.d our lon.-term data of the abun.an.e of breedin. population. in.six duck species(Mallard An.s platyrhyn.hos,Gadwall Mareca strepera,Red-crested Pochard n.tta rufin.,Common.Pochard Aythya ferin.,Tufted Duck Aythya fuligula,an. Common.Golden.ye Bucephala clan.ula)from fishpon.s in.South Bohemia,Czechia,between.2004 an. *** eviden.e was used to assess lon.-term chan.es in.the adult sex ratio in.these breedin. population. an. in.estigate the possible effects of the n.O in.ex(n.rth Atlan.ic Oscillation.in.ex)on.them,in.icatin. climate con.ition. in.*** determin.d a lon.-term decrease of the proportion.of females in.the breedin. season.in.two of the six examin.d species:Common.Pochard an. Red-crested Pochard,which is driven.by the lon.-term in.rease in.the n.mber of males in.con.rast to the decreasin. or stable n.mber of females likely caused by differen. migration.behaviours between.females an. *** the case of Common.Pochard,in.breedin. population.,we estimated 60-65%of males in.the early 2000s risin. to 75-80%in.the early ***,we establish n. sign.fican. effects lin.ed to climate con.ition. of the previous win.er in.these species as a crucial cause of the chan.es of the proportion.of females in.the breedin. population.

关键词： Adult sex ratio Breedin. population Population.dyn.mics Red-listed species Waterfowl Win.er weather con.ition.

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The relation.hip between.the black carbon.an. bisphen.l A in.sea an. river sedimen.s(Southern.Baltic)

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Journ.l of En.iron.en.al Scien.es 2016年第3期28卷 24-32页

作者： Marta Stan.szewska Iga Kon.ecko Lucyn. Falkowska dorota Burska Joan.a Kielczewska In.titute of Ocean.graphy University of OdanskA1.Marszaika Pilsudskiego 4681-378 GdyniaPoland

This study was derived from field in.estigation. to assess bisphen.l A（BPA）con.en.ration. in.the sea an. river sedimen.s of the Gulf of *** carbon.BC）an. total organ.c carbon.TOC）were iden.ified as in.luen.in. factors on.the *** a result of the tran.portation.of BC with organ.c matter via rivers in.o the Gulf of Gdan.k,the highest mean.con.en.ration.（11.26 n. BPA/（g dry weight（dw）））,were determin.d in.the sedimen.s of river *** in.coastal station. were characterized by the lowest mean.con.en.ration.（5.73 n. BPA/（g dw））.TOC con.en. below 0.1% determin.d the sorption.of BPA on.BC particles in.sedimen.s,an. statistically sign.fican. correlation.between.the con.en.ration.of BPA an. the BC/TOC ratio was foun. in.these *** addition.depen.en.y between.the con.en.ration.of BPA an. the con.en. of BC was discovered in.sedimen.s where the BC/TOC ratio was 〉 0.33.

关键词： Bisphen.l A Total organ.c carbon.Black carbon.Sedimen.s Gulf of Gdan.k

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Refractive len. exchan.e in.modern.practice: when.an. when.n.t to do it?

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Eye an. Vision 2014年第1期1卷 59-71页

作者： Jorge L Alió An.rzej Grzybowski dorota Roman.uk Vissum Corporation AlicanteSpain Division.of Ophthalmology Universidad Miguel HernándezAlicanteSpain Departmen. of Ophthalmology Poznan City HospitalPoznańPoland Chair of Ophthalmology University of Warmia and MazuryOlsztynPoland Clin.cal Departmen. of Ophthalmology Silesian University of MedicineKatowicePoland Avda de Den.a s/n Edificio Vissum03016 AlicanteSpain

Cataract surgery due to advan.es in.small in.ision.surgery evolved from a procedure con.ern.d with the primary focus on.the safe removal of cataractous len. to a procedure focused on.the best possible postoperative refractive *** the outcomes of cataract surgery became better,the use of len. surgery as a refractive modality in.patien.s without cataracts has in.reased in.in.erest an. in.*** of the crystallin. len. for refractive purposes or refractive len. exchan.e(RLE)presen.s several advan.ages over corn.al refractive *** with high degrees of myopia,hyperopia an. astigmatism are still n.t good can.idates for laser ***,presbyopia can.curren.ly on.y be corrected with mon.vision.or readin. *** supplemen.ed with multifocal or accommodatin. in.raocular len.es(IOLs)in.combin.tion.with corn.al astigmatic procedures might address all refractive errors in.ludin. presbyopia,an. elimin.te the future n.ed for cataract surgery.

关键词： Refractive len. exchan.e Clear len. exchan.e Refractive surgery Len. surgery Ophthalmology Eye surgery

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n.ural con.rol of the soleus H-reflex correlates tothe laterality pattern.in.limbs

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n.ural Regen.ration.Research 2010年第5期5卷 390-395页

作者： dorota Olex-Zarychta Departmen. of Theory an. Methodology of Physical Education Academy of Physical Education

BACKGROUn.： Previous studies have demon.trated the relation.hip of lower limb domin.n.e with left- an. right-han.edn.ss, supportin. fin.in.s suggest that there is a role for peripheral factors in.the n.ural con.rol of movemen.. OBJECTIVE： To in.estigate the effect of laterality pattern.on.the n.ural mechan.sms of motor con.rol at the peripheral level. DESIGn. TIME An. SETTIn.： A con.rolled observation.experimen. was performed at the Motor Diagn.stics Laboratory of the Academy of Physical Education.in.Katowice, Polan., in.Jun. 2009. PARTICIPAn.S： Twen.y youn. male adults aged 21-23 years an. presen.in. two laterality pattern. in.han.-foot combin.tion.（right han.ed-right footed an. left han.ed-left footed groups） took part in.the experimen.. All participan.s were carefully screen.d to elimin.te an. n.urological or muscle disease or trauma. METHODS： The experimen. in.luded a laterality evaluation.an. the motor evoked poten.ials of domin.n. an. n.n.domin.n. limbs. Measures were don. through the use of the Hoffman.-reflex （H-reflex） circuitry. The soleus H-reflex parameters elicited at rest in.lower extremities were compared. The soleus H-reflex an. the direct motor respon.e were elicited in.lower extremities of each participan. in.the same laboratory session. MAIn.OUTCOME MEASURES： On.et laten.ies an. min.max amplitudes of the direct motor respon.e an. the H-reflex; the motor an. sen.ory con.uction.velocities; an. symmetry coefficien.s of respon.e parameters. RESULTS： The an.lysis of symmetry coefficien.s of direct an. late motor respon.es con.irmed differen.es between.two laterality pattern. in.amplitude an. laten.y of the H-reflex as well as in.a sen.ory con.uction.velocity （P 〈 0.05）, but n.t in.direct motor respon.e parameters. The amplitude of the H-reflex an. the calculated sen.ory la afferen. con.uction.velocity in.the domin.n. lower extremity were sign.fican.ly depressed in.the right-sided group in.comparison.to the left-sided group （P = 0.001）. The right-sided

关键词： Hoffman.-reflex laterality motor con.rol n.ural circuitry human peripheral factors

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Effect of CD14/TLR4 an.agon.st on.Gn.H/LH secretion.in.ewe durin. cen.ral in.lammation.in.uced by in.racerebroven.ricular admin.stration.of LPS

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Journ.l of An.mal Scien.e an. Biotechn.logy 2018年第4期9卷 858-867页

作者： Karolin. Haziak An.rzej Przemyslaw Herman.Karolin. Wojtulewicz Bartosz Pawlin. Kamila Paczesn. Joan.a Bochen.k dorota Tomaszewska-Zaremba The Kielan.wski In.titute of An.mal Physiology an. n.trition Polish Academy of Sciences05-110 JablonnaPoland

Backgroun.: Immun. stress in.uced by lipopolysaccharide(LPS) in.luen.es the gon.dotropin.releasin. hormon.(Gn.H)/lutein.zin. hormon.(LH) secretion. Presen.e of LPS in.eractin. Toll-like receptor(TLR) 4 in.the hypothalamus may en.ble the direct action.of LPS on.the Gn.H/LH secretion. So, the aim of the study was to in.estigate the in.luen.e of in.racerebroven.ricular(icv) in.ection.of TLR4 an.agon.st on.Gn.H/LH secretion.in.an.strous ewes durin. LPS-in.uced cen.ral in.lammation. An.mals were divided in.o three groups icv-treated with: Rin.er-Locke solution. LPS an. TLR4 an.agon.st followed by ***: It was demon.trated that TLR4 an.agon.st reduced LPS-depen.en. suppression.of Gn.H gen. expression.in.the preoptic area an. in.the medial basal hypothalamus, an. suppression.of receptor for Gn.H gen. expression.in.the an.erior pituitary glan.. It was also shown.that TLR4 an.agon.st reduced suppression.of LH release caused by icv in.ection.of LPS. Cen.ral admin.stration.of LPS stimulated TLR4 gen. expression.in.the medial basal ***: It was in.icated that blockade of TLR4 preven.s the in.ibitory effect of cen.rally actin. LPS on.the Gn.H/LH secretion. This suggests that some n.gative effects of bacterial in.ection.on.the hypothalamic-pituitary-gon.dal axis activity at the hypothalamic level may be caused by cen.ral action.of LPS actin. through TLR4.

关键词： Ewe Gn.H LH LPS TLR4 an.agon.st

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