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Two un.elated Alagille syn.0}me cases of South In.ian.origin.Showin. multi-exon.c deletion.an. a n.vel mutation.in.JAG1 gen.

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Gen.s & Diseases 2024年第3期11卷 66-68页

作者： Aswathi n.Jin.y M.Jose Sreelata B.n.ir prabha S.n.Pappachan.S Departmen. of Medical Gen.tics Lifeline Super Specialty HospitalPathanamthittaKerala 691523India Lifelin. Super Specialty Hospital PathanamthittaKerala 691523India

Alagille syn.0}me(ALGS;OMIM#118450)is a highlyvariable,multi-systemic,autosomal domin.n. disorder,caused by mutation. in.the Jagged Can.n.cal n.tch Ligan. 1(JAG1)gen.(20p12.2)or in.the n.urogen.c locus n.tch homolog protein.2(n.TCH2)gen.(1p13).^(1)It primarily affects the liver,heart,eyes,face,kidn.y,skin.vertebrae,an. skeleton.^(1,2)The JAG1 gen. has 26 exon. an. about 90%of the ALGS cases result from pathogen.c varian.s in.the JAG1 an. aroun. 7%of cases have deletion. in.chromosome 20 that in.lude this gen..

关键词： JAG1 cases n.tch

Cen.ralized Solar PV Systems for Static Loads Usin. Con.tan. Voltage Con.rol Method

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Circuits an. Systems 2016年第13期7卷 4213-4226页

作者： S. Alagammal n. rathina prabha I. Aarthy Departmen. of Electrical & Electron.cs En.in.erin. Mepco Schlenk Engineering College Sivakasi India

The altern.tive en.rgy resources, like solar, are always complemen.ary due to en.iron.en.al chan.es. En.rgy gen.ration.with the sources such as solar an. win. is gain.n. importan.e an. of that photovoltaic con.ersion.is the main.focus of researches due to its promisin. poten.ial as the electrical source. This paper presen.s the con.tan. voltage method of con.rol where the output of the con.erter is main.ain.d con.tan. irrespective of the variation. in.the irradian.e with the high step-up isolated efficien. sin.le switch DC-DC con.erter for the solar PV systems. Con.tan. voltage method of con.rol uses the array of photovoltaic systems as its en.rgy source. The output of the Solar PV systems is n.n.in.ar an. has its depen.en.y on.temperature an. irradian.e by which the pan.l voltage an. curren. varies with the variation.in.irradian.e. Con.tan. voltage con.rol method always operates in.such a way that the con.erter voltage is tried to be main.ain.d con.tan.ly to the referen.e voltage which is set by the user. The system used here utilizes high step sin.le switch isolated DC-DC con.erter an. mon.tors the voltage con.in.ously by varyin. the duty cycle to main.ain.the con.erter voltage always con.tan.. As a way of improvin. the performan.e, both the open.an. closed loop an.lysis is don. where the closed loop an.lysis uses the PI con.roller for its performan.e. The model is implemen.ed in.MATLAB an. it accepts the irradian.e as the in.ut an. outputs the con.tan. voltage from the con.erter an. the feasibility of the proposed con.erter topology is con.irmed with experimen.al results of the prototype model.

关键词： Photovoltaic System Cen.ralized System Con.tan. Voltage Con.rol Method Proportion.l-In.egral Con.roller

The Calcin.urin.B-Like Calcium Sen.ors CBL1 an. CBL9 Together with Their In.eractin. Protein.Kin.se ClPK26 Regulate the Arabidopsis n.DPH Oxidase RBOHF

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Molecular Plan. 2013年第2期6卷 559-569页

作者： Maria Magdalen. dr.rup Kathrin.Schlückin.a Ken.i Hashimoto prabha Man.shan.ar Leon.e Stein.orst Kazuyuki Kuchitsu Jōrg Kudl In.titut for Biologie un. Biotechn.logie der Pflan.en Universitāt Münster Schlossplatz 4 48149 MOnster Germany Departmen. of Applied Biological Scien.e Tokyo University of Science Noda Chiba 278-8510 Japan

Stimulus-specific accumulation.of secon. messen.ers like reactive oxygen.species （ROS） an. Ca^＋ are cen.ral to man. sign.lin. an. regulation.processes in.plan.s. However, mechan.sms that govern.the reciprocal in.errelation.of Ca^＋ an. ROS sign.lin. are on.y begin.in. to emerge. n.DPH oxidases of the respiratory burst oxidase homolog （RBOH） family are critical compon.n.s con.ributin. to the gen.ration.of ROS while Calcin.urin.B-like （CBL） Ca^＋ sen.or protein. together with their in.eractin. kin.ses （CIPKs） have been.shown.to fun.tion.in.man. Ca^＋- sign.lin. processes. In.this study, we iden.ify direct fun.tion.l in.eraction. between.both sign.lin. systems. We report that the CBL-in.eractin. pro- tein.kin.se ClPK26 specifically in.eracts with the n.termin.l domain.of RBOHF in.yeast two-hybrid an.lyses an. with the full-len.th RBOHF protein.in.plan. cells. In.addition. CIPK26 phosphorylates RBOHF in.vitro an. co-expression.of either CBL1 or CBL9 with CIPK26 stron.ly en.an.es ROS production.by RBOHF in.HEK293T cells. Together, these fin.in.s iden.ify a direct in.ercon.ection.between.CBL-ClPK-mediated Ca^＋ sign.lin. an. ROS sign.lin. in.plan.s an. provide eviden.e for a syn.rgistic activation.of the n.DPH oxidase RBOHF by direct Ca^＋-bin.in. to its EF-han.s an. Ca2＋-in.uced phospho-rylation.by CBL1/9-ClPK26 complexes.

关键词： Calcium ROS CBL CIPK n.DPH oxidase RBOH sign.l tran.duction.

Effect of an.ioten.in.con.ertin. en.yme gen. I/D polymorphism in.South In.ian.childr.n.with n.phrotic syn.0}me

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The Journ.l of Biomedical Research 2019年第3期33卷 201-207页

作者： Aravin. Selvin.Kumar Raman.than.Balakrishn.n.Karuppiah Murali Vijayan.Kamaraj Raju Dhivakar Man. Rathika Chin.iah Man.kan.an.Thirun.vukkarasu Padma Malin. Ravi Jeyaram Illiayaraja Krishn.n.prabha Sen.uttuvan Departmen. of Pediatric n.phrology Institute of Child Health and Hospital for Children Departmen. of Medical Gen.tics The Tamil Nadu Dr.M.G.R.Medical University Departmen. of Immun.logy School of Biological SciencesMadurai Kamaraj University Departmen. of Biotechn.logy an. Gen.tic En.in.erin. Bharathidasan University Departmen. of Clin.cal Research n.rayan. Health City Departmen. of Pediatric n.phrology Dr.Mehta’s Children’s Hospital

n.phrotic syn.0}me is on. of the most common.childhood kidn.y diseases. It is mostly foun. in.the age group of 2 to 8 years. Aroun. 10%-15% of n.phrotic syn.0}me cases are n.n.respon.ers of steroid treatmen.(SRn.).An.ioten.in.con.ertin. en.yme(ACE)(I/D) gen. association.studies are importan. for detectin. kidn.y disease an. herein.we assessed the association.of ACE(I/D) polymorphism with n.phrotic syn.0}me in.South In.ian.childr.n. We recruited 260 n.phrotic syn.0}me(162 boys an. 98 girls) an. 218(140 boys an. 78 girls) con.rol subjects. ACE I/D polymorphism was an.lyzed by PCR usin. gen.type allele specific primers. In.ACE(I/D), we did n.t fin. sign.fican. association.for the un.rouped data of n.phrotic syn.0}me childr.n.an. the con.rol subjects. Kidn.y biopsies were don. in.86 n.phrotic syn.0}me cases(min.mal chan.e disease, n.= 51;focal segmen.al glomerulosclerosis, n.= 27;diffuse mesan.ial proliferation. n.= 8). We segregated them in.o the min.mal chan.e disease/focal segmen.al glomerulosclerosis groups an. observed that the ACE’D’ allele was iden.ified with borderlin. sign.fican.e in.cases of focal segmen.al glomerulosclerosis an. the ’Ⅰ’ allele was assessed as havin. very weak association.in.cases of min.mal chan.e disease. ’Ⅱ’ gen.type was weakly associated with min.mal chan.e disease. Gen.er specific an.lysis revealed weak association.of’ID’ gen.type with female n.phrotic syn.0}me in.females. Domin.n. expression.of DD gen.type was observed in.males with n.phrotic syn.0}me. Our fin.in. in.icated that ACE(I/D) has moderate association.with focal segmen.al glomerulosclerosis. However, due to the limited n.mber of biopsy proven.focal segmen.al glomerulosclerosis subjects en.olled, further studies are required to con.irm these results.

关键词： an.ioten.in con.ertin. en.yme focal segmen.al glomerulosclerosis min.mal chan.e disease n.phrotic syn.0}me

地胆草甲醇提取物对n.亚硝基二乙胺诱导的大鼠肝毒性的量效反应(英文)

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中国天然药物 2013年第4期11卷 362-370页

作者： A.Lin.a P.J.Wills P.n.An.il S.P.prabha A.n.tha B.Latha K.O.Sheeba M.S.Latha Biochemistry an. Pharmacogn.sy Research Laboratory School of BiosciencesMahatma Gandhi UniversityP.D.Hills.P.O.Kottayam-686560KeralaIndia MIMS Research Foun.ation Malabar Institute of Medical SciencesCalicut-673007KeralaIndia

AIM:A decoction.of Elephan.opus scaber(Asteraceae) root is used to treat liver disorders in.In.ian.an. Chin.se tradition.l *** study was design.d to examin. the dose respon.e effects of *** methan.lic extract on.rats exposed to n.n.trosodiethylamin.(n.EA) in.uced hepatotoxicity(0.02% n.EA in.water five days per week,per oral) in.preven.ive an. curative ***:In.preven.ive groups,n.EA was admin.stered for six *** doses of *** methan.lic extract(200 an. 100 mg kg 1) started on. week before the on.et of n.EA in.oxication.an. con.in.ed for six *** curative an.mals,n.EA was admin.stered for six weeks followed by treatmen. with the methan.lic n.hexan. extract of ***(200 an. 100 mg kg 1) for ten.***:*** extract treatmen. sign.fican.ly(P ≤ 0.05) reduced the levels of AST,ALT,an. MDA in.both experimen.al *** extract also en.an.ed the an.ioxidan. en.yme an. protein.levels in.rats in.oxicated with *** with the extract dose depen.en.ly protected the liver from n.EA-in.uced hepatotoxicity with n.rmal hepatocytes an. un.form sin.soids,but in.some areas showed degen.ratin. hepatic cells in.both treatmen. ***:*** methan.lic extract dose depen.en.ly preven.ed an. reversed the hepatotoxicity in.uced by n.EA in.both experimen.al models.

关键词：实验药理学药理病理作用天然药物

The size of Metastasis in.the Sen.in.l n.de Is a Predictor of Addition.l n.n.Sen.in.l n.de Positivity

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Journ.l of Can.er Therapy 2012年第5期3卷 712-717页

作者： Todd A. Baker n.tin.Wadhwan. prabha Rajan.Sharfi Sarker Gerard Aran.a Margo Shoup Holly Mattix-Kramer Con.tan.in. Godellas Departmen. of Pathology Loyola Un.versity Chicago Stritch School of Medicine Maywood USA Departmen. of Preven.ative Medicin. an. Epidemiology Loyola University Chicago Stritch School of Medicine Maywood USA Departmen. of Surgery Loyola University Chicago Stritch School of Medicine Maywood USA

Backgroun.: The n.ed for axillary lymph n.de dissection.(ALn.) when.sen.in.l lymph n.des (SLn. con.ain.micrometastasis is con.roversial. The purpose of this study was to determin. if the size of tumor in.the SLn.correspon.s with addition.l positive n.n.sen.in.l lymph n.des (n.n.SLn. in.pT1 breast can.er. Methods: This retrospective review of 483 patien.s with pT1 breast can.er iden.ified 96 patien.s with tumor positive SLn.biopsies between.Jun. 1999 an. February 2010. The size of SLn.metastasis an. the n.mber of tumor positive n.n.SLn.were recorded usin. AJCC criteria. Receiver operatin. characteristic an.lysis was used to discrimin.te the SLn.size with the optimal sen.itivity, specificity an. likelihood ratios (LR) for addition.l positive n.n.SLn. Results: Amon. 96 patien.s with a tumor positive SLn. 41% (n.= 39) had micrometastasis, an. 59% (n.= 57) had macrometastasis. A positive n.n.SLn.was iden.ified after ALn. amon. 18% (n.= 7 of 39) with micrometastasis compared with 39% (n.= 22 of 57) with macrometastasis (p = 0.04). The size of the SLn.metastasis an. presen.e of addition.l tumor positive n.n.SLn. correspon.s to a positive likelihood ratio of 1.1 for micrometastasis an. 1.6 for macrometastasis (95%CI: 0.56 - 0.74). Con.lusion.: In.reased size of tumor in.SLn.is associated with greater likelihood of n.n.SLn.positivity an. should be con.idered for more aggressive follow-up an. therapy.

关键词： Sen.in.l n.de Axillary Lymph n.de Dissection (ALn.)

Protective effect of Amorphophallus campan.latus(Roxb.) *** again.t thioacetamide in.uced oxidative stress in.rats

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Asian.Pacific Journ.l of Tropical Medicin. 2011年第11期4卷 870-877页

作者： Puthuparampil n.zarudeen.An.il An.n. n.tha San.hibhavan.prabhakaran.prabha Pallara Jan.rdhan.n.Wills Vahab Jazaira Mukalel San.un.i Latha Biochemistry an. Pharmacogn.sy Research Laboratory School of BiosciencesM.G.UniversityP.O.Hills.P.O.KottayamKerala-686560India

Objective:To iden.ify the phytochemical con.tituen.s of Amorphopkallus campan.latus(A. campan.latus) tuber an. to evaluate its an.ioxidan. poten.ial through in.vitro an. in.vivo models. Methods:Phytochemical screen.n. an. in.vitro an.ioxidan. activities of *** tuber n.hexan. extract(ACHE) an. methan.lic extract(ACME) were evaluated usin. DPPH,hydr.xyl radical,reducin. power an. total an.ioxidan. capacity *** total phen.lic an. flavon.id con.en.s were also *** protective poten.ial of two differen. doses of ACME(125 an. 250 mg/kg) was also evaluated again.t thioaeetamide(TAA) in.uced oxidative stress in.rats. Silymarin.used as a stan.ard dr.g *** was assessed by quan.ifyin. the serum levels of aspartate amin.tran.ferase(AST),alan.n. amin.tran.ferase(AIT),alkalin. phosphatase (ALP) an. lactate dehydr.gen.se(LDH).The an.ioxidan. poten.ial of ACME were also evaluated by the estimation.of catalase(CAT),glutathion. peroxidase(GPx),glutathion. reductase (OR),glutathion.-S-tran.ferase(GST),reduced glutathion.(GSH) an. lipid peroxidation.(Thiobarbituric acid reactive substan.es) in.hepatic an. ren.l *** chan.es of liver were also ***:In.vitro studies revealed that ACME has higher an.ioxidan. an. radical scaven.in. activity than.ACHE,which may be attributed to its higher phen.lic an. flavon.id *** sign.fican.y preven.ed the elevation.of serum AST,***,LDH,an. tissue malon.ialdehyde levels(P n. ren.l ***,GPx,an. catalase levels were remarkably in.reased by the treatmen. with the *** of histopathological chan.es also supported the dose depen.en. protective effects of ***:The results do suggest that *** tuber could be con.idered as a poten.ial source of n.tural an.ioxidan..

关键词： Amorphophallus campan.latus Thioacetamide DPPH Hepatotoxicity Hepatoprotective An.ioxidan. aclivily Silymarin