作者：Arumugam Ramakrishnan ; Natesan Vijayakumar ; ^{nvkbiochem@yahoo.co.in} ; Mani Renuka

关键词：NH4Cl ; ammonium chloride ; i.p ; intraperitonial injection ; b.w. ; body weight ; NO ; nitric oxide ; CPS-1 ; carbamoylphosphate synthetase-1 ; OTC ; ornithine transcarbamoylase ; ASS ; argininosuccinate synthetase ; ASL ; argininosuccinate lyase ; CNS ; central nervous system ; Na+/K+ ATPase ; sodium/potassium ATPase ; NH3 ; ammonia ; GS ; glutamine synthetase ; NOS ; nitric oxide synthase ; NMDA ; N-methyl-d-aspartic acid ; cGMP ; cyclic guanosine monophosphate ; sGC ; soluble guanylate cyclase ; ROS/RNS ; reactive oxygen sp

刊名：Biomedicine & Pharmacotherapy

出版年：2016

作者：Thomas Opladen^a ; ^{Thomas.Opladen@med.uni-heidelberg.de" class="auth_mail" title="E-mail the corresponding author} ; Martin Lindner^a ; ^b ; ^{Martin.Lindner@kgu.de" class="auth_mail" title="E-mail the corresponding author} ; Anibh M. Das^c ; ^{Das.Anibh@mh-hannover.de" class="auth_mail" title="E-mail the corresponding author} ; Thorsten Marquardt^d ; ^{Thorsten.Marquardt@ukmuenster.de" class="auth_mail" title="E-mail the corresponding author} ; Aneal Khan^e ; ^{khaa@ucalgary.ca" class="auth_mail" title="E-mail the corresponding author} ; Sukru H. Emre^f ; ^{sukru.emre@yale.edu" class="auth_mail" title="E-mail the corresponding author} ; Barbara K. Burton^g ; ^{BBurton@luriechildrens.org" class="auth_mail" title="E-mail the corresponding author} ; Bruce A. Barshop^h ; ^{bbarshop@ucsd.edu" class="auth_mail" title="E-mail the corresponding author} ; Thea Bö ; hmⁱ ; ^{Thea.boehm@gmx.net" class="auth_mail" title="E-mail the corresponding author} ; Jochen Meyburg^a ; ^{Jochen.Meyburg@med.uni-heidelberg.de" class="auth_mail" title="E-mail the corresponding author} ; Kathrin Zangerl^a ; ^{kathrin.zangerl@gmail.com" class="auth_mail" title="E-mail the corresponding author} ; Sebene Mayorandan^c ; ^{Sebene.Mayorandan@ukmuenster.de" class="auth_mail" title="E-mail the corresponding author} ; Peter Burgard^a ; ^{Peter.Burgard@med.uni-heidelberg.de" class="auth_mail" title="E-mail the corresponding author} ; Ulrich H.N. Dü ; rrⁱ ; ^{Ulrich.Duerr@cytonet.de" class="auth_mail" title="E-mail the corresponding author} ; Bernd Rosenkranz^j ; ^{rosenkranz@sun.ac.za" class="auth_mail" title="E-mail the corresponding author} ; Jö ; rg Renneckeⁱ ; ^{Joerg.Rennecke@cytonet.de" class="auth_mail" title="E-mail the corresponding author} ; Jens Derbinskiⁱ ; ^{Jens.Derbinski@cytonet.de" class="auth_mail" title="E-mail the corresponding author} ; Marc Yudkoff^k ; ¹ ; ^{yudkoff@email.chop.edu" class="auth_mail" title="E-mail the corresponding author} ; Georg F. Hoffmann^a ; ¹ ; ^{Georg.Hoffmann@med.uni-heidelberg.de" class="auth_mail" title="E-mail the corresponding author}

关键词：UCD ; urea cycle defect ; OTC ; ornithine carbamoyltransferase ; ASS ; argininosuccinate synthetase 1 ; CPS1 ; carbamoyl phosphate synthetase 1 ; ASL ; argininosuccinate lyase

刊名：Molecular Genetics and Metabolism

出版年：2016

作者：Vijayakumar Natesan ; ^{nvkbiochem@yahoo.co.in" class="auth_mail" title="E-mail the corresponding author} ; Renuka Mani ; Ramakrishnan Arumugam

关键词：N2 ; nitrogen ; NH3 ; ammonia ; Ca2+ ; calcium ; CPS-1 ; carbomyl phosphate synthetase-1 ; OTC ; ornithine transcarbomylase ; ASS-1 ; argininosuccinate synthetase-1 ; ORNT ; ornithine transporter ; HHH ; hyperornithemia ; hyperammonemia ; homocitrullinemia syndrome ; ASL ; argininosuccinate lyase ; GABA ; γ-amino-butyric acid ; CNS ; central nervous system ; mPT ; mitochondrial permeability transition ; HE ; hepatic encephalopathy ; ONS ; oxidative/nitrosative stress ; PTP ; permeability transition pore ; NMDA ; N-methyl-d-aspa

刊名：Biomedicine & Pharmacotherapy

出版年：2016

作者：Zhu Huang^a ; ^d ; Tong-Song Wang^b ; Yue-Chao Zhao^c ; Ru-Juan Zuo^a ; Wen-Bo Deng^a ; Yu-Jing Chi^c ; Zeng-Ming Yang^b ; ^e ; ^{zmyang@scau.edu.cn" class="auth_mail}

关键词：Argininosuccinate synthase 1 ; cAMP ; Decidualization ; Uterus

刊名：Molecular and Cellular Endocrinology

出版年：5 May, 2014

作者：Wendy Smith ; MD¹ ; ^{SMITHW@mmc.org} ; George A. Diaz ; MD ; PhD² ; Uta Lichter-Konecki ; MD ; PhD³ ; Susan A. Berry ; MD⁴ ; Cary O. Harding ; MD⁵ ; Shawn E. McCandless ; MD⁶ ; Cindy LeMons⁷ ; Joe Mauney ; MS⁸ ; Klara Dickinson ; BS⁹ ; Dion F. Coakley ; PharmD⁹ ; Tristen Moors ; MS⁹ ; Masoud Mokhtarani ; MD⁹ ; Bruce F. Scharschmidt ; MD⁹ ; Brendan Lee ; MD ; PhD¹⁰ ; ¹¹

关键词：ASA ; Argininosuccinic acid ; ASL ; Argininosuccinate lyase ; ASS ; Argininosuccinate synthetase ; AUC0-24 ; 24-hour area under the curve ; DSMB ; Data and safety monitoring board ; G tube ; Gastrostomy tube ; GPB ; Glycerol phenylbutyrate ; NaPBA ; Sodium phenylbutyrat

刊名：The Journal of Pediatrics

出版年：2013

作者：Shao-Ju Chien ; Kuan-Miao Lin ; Hsuan-Chang Kuo ; Chien-Fu Huang ; Ying-Jui Lin ; Li-Tung Huang ; You-Lin Tain

关键词：ADMA ; asymmetric dimethylarginine ; ASL ; argininosuccinate lyase ; ASS ; argininosuccinate synthetase ; BP ; blood pressure ; CAT ; cationic amino acid transporter ; DDAH ; dimethylarginine dimethylaminohydrolase ; eNOS ; endothelial nitric oxide synthase ; EPR ; electron paramagnetic resonance ; IOD ; integrated optical density ; NO ; nitric oxide ; NOS ; nitric oxide synthase ; nNOS ; neuronal nitric oxide synthase ; OHdG ; 8-hydroxydeoxyguanosine ; ROS ; reactive oxygen species ; SHR ; spontaneously hypertensive rat ; W

刊名：Translational Research

出版年：January, 2014

作者：Susan A. Berry^a ; ^{berry002@umn.edu" class="auth_mail} ; Uta Lichter-Konecki^b ; George A. Diaz^c ; Shawn E. McCandless^d ; William Rhead^e ; Wendy Smith^f ; Cynthia LeMons^g ; Sandesh C.S. Nagamani^h ; Dion F. Coakleyⁱ ; Masoud Mokhtaraniⁱ ; Bruce F. Scharschmidtⁱ ; Brendan Lee^h

关键词：ASL ; argininosuccinate lyase ; ASS ; argininosuccinate synthase ; AUC0&ndash ; 24 ; 24  ; hour area under the curve ; GPB ; glycerol phenylbutyrate (generic name for glyceryl tri (4-phenylbutyrate) ; also referred to as HPN-100 or RAVICTI®) ; NaPBA ; sodium phenylbutyrate ; OTC ; ornithine transcarbamylase ; PAA ; phenylacetic acid ; PAGN ; phenylacetylglutamine ; PBA ; phenylbutyric acid ; PK ; pharmacokinetic ; UCD ; urea cycle disorder

刊名：Molecular Genetics and Metabolism

出版年：May 2014

作者：Nicholas Ah Mew ; MD¹ ; ² ; ^{nahmew@cnmc.org} ; Lauren Krivitzky ; PhD³ ; Robert McCarter ; ScD¹ ; ² ; Mark Batshaw ; MD¹ ; ² ; Mendel Tuchman ; MD¹ ; ² ; Urea Cycle Disorders Consortium of the Rare Diseases Clinical Research Network &lowast

关键词：ALD ; Argininosuccinate lyase deficiency ; ASD ; Argininosuccinate synthetase deficiency ; CPSD ; Carbamyl phosphate synthetase 1 deficiency ; DQ ; Developmental quotient ; FSIQ ; Full-scale intelligence quotient ; OTC ; Ornithine transcarbamylase ; OTCD ; Ornithine t

刊名：The Journal of Pediatrics

出版年：2013

作者：Ya-Huei Chen^a ; ¹ ; Ming-Ting Lee^a ; ¹ ; Yu-Wen Cheng^a ; Wei-Yuan Chou^a ; Chung-Ming Yu^b ; Hwei-Jen Lee^a ; ^{hjlee@mail.ndmctsgh.edu.tw}

关键词：α ; A-Crystallin ; Molecular chaperone ; Goose δ ; -crystallin ; Argininosuccinate lyase ; Thermal stability ; Protein aggregate

刊名：Biochimie

出版年：2011

作者：Yi-Yu Wei^a ; Chih-Wei Huang^b ; Wei-Yuan Chou^a ; Hwei-Jen Lee^a ; ^{hjlee@mail.ndmctsgh.edu.tw}

关键词：Argininosuccinate lyase ; Cold labile ; Freeze&ndash ; thawing ; Ice-induced inactivation ; ¦Á-Crystallin ; Cryoprotection

刊名：Biochimie

出版年：2012