関: argininosuccinate

WordNet

street name for lysergic acid diethylamide (同)back breaker, battery-acid, dose, dot, Elvis, loony toons, Lucy in the sky with diamonds, pane, superman, window pane, Zen
any of various water-soluble compounds having a sour taste and capable of turning litmus red and reacting with a base to form a salt
having the characteristics of an acid; "an acid reaction"

PrepTutorEJDIC

酸性の / 酸味のある,すっぱい(sour) / (言葉・態度などが)厳しい,しんらつな / 酸 / すっぱいもの / 《俗》=LSD

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/12/08 01:26:31」(JST)

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Argininosuccinic acid is a chemical compound that is a basic amino acid.

Reactions

Some cells synthesize argininosuccinic acid from citrulline and aspartic acid and use it as a precursor for arginine in the urea cycle or citrulline-NO cycle. The enzyme that catalyzes the reaction is argininosuccinate synthetase.

Argininosuccinic acid is a precursor to fumarate in the citric acid cycle via argininosuccinate lyase.

Synonyms

Argininosuccinate

UpToDate Contents

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1. 尿素サイクル障害：管理 urea cycle disorders management
2. 尿素サイクル障害：臨床的特徴および診断 urea cycle disorders clinical features and diagnosis
3. 胎児の酸塩基の生理学 fetal acid base physiology
4. 神経管欠損予防のための葉酸 folic acid for prevention of neural tube defects
5. 疾患予防におけるビタミン補給 vitamin supplementation in disease prevention

English Journal

Ammonia Control in Children Ages 2 Months through 5 Years with Urea Cycle Disorders: Comparison of Sodium Phenylbutyrate and Glycerol Phenylbutyrate.

Smith W, Diaz GA, Lichter-Konecki U, Berry SA, Harding CO, McCandless SE, Lemons C, Mauney J, Dickinson K, Coakley DF, Moors T, Mokhtarani M, Scharschmidt BF, Lee B.SourceDivision of Genetics, Department of Pediatrics Maine Medical Center, Portland, ME. Electronic address: SMITHW@mmc.org.
The Journal of pediatrics.J Pediatr.2013 Jun;162(6):1228-1234.e1. doi: 10.1016/j.jpeds.2012.11.084. Epub 2013 Jan 13.
OBJECTIVES: To examine ammonia levels, pharmacokinetics, and safety of glycerol phenylbutyrate (GPB; also referred to as HPN-100) and sodium phenylbutyrate (NaPBA) in young children with urea cycle disorders (UCDs).STUDY DESIGN: This open label switch-over study enrolled patients ages 29 days to und
PMID 23324524

A randomized controlled trial to evaluate the effects of high-dose versus low-dose of arginine therapy on hepatic function tests in argininosuccinic aciduria.

Nagamani SC, Shchelochkov OA, Mullins MA, Carter S, Lanpher BC, Sun Q, Kleppe S, Erez A, O'Brian Smith E, Marini JC; Members of the Urea Cycle Disorders Consortium, Lee B.Collaborators (20)Batshaw ML, Tuchman M, Summar ML, Baumgartner MR, Berry SA, Cederbaum S, Diaz GA, Gallagher RC, Harding CO, Hoffmann G, Kerr DS, Lichter-Konecki U, McCandless SE, Merritt J, Schulze A, Seashore MR, Stricker T, Waisbren S, Wong D, Yudkoff M.
Molecular genetics and metabolism.Mol Genet Metab.2012 Nov;107(3):315-21. doi: 10.1016/j.ymgme.2012.09.016. Epub 2012 Sep 17.
OBJECTIVE: To compare the effects of combinatorial therapy with low-dose arginine and a nitrogen scavenging agent (sodium phenylbutyrate) vs. monotherapy with high-dose arginine on liver function tests in patients with argininosuccinic aciduria (ASA).STUDY DESIGN: Twelve patients with ASA were enrol
PMID 23040521

Dietary management of urea cycle disorders: UK practice.

Adam S, Champion H, Daly A, Dawson S, Dixon M, Dunlop C, Eardley J, Evans S, Ferguson C, Jankowski C, Lowry S, MacDonald A, Maritz C, Micciche A, Robertson L, Stafford J, Terry A, Thom R, van Wyk K, Webster D, White FJ, Wildgoose J; British Inherited Metabolic Diseases Group (BIMDG) Dietitian’s Group.SourceRoyal Hospital for Sick Children Glasgow, Glasgow Royal Infirmary, Glasgow, UK.
Journal of human nutrition and dietetics : the official journal of the British Dietetic Association.J Hum Nutr Diet.2012 Aug;25(4):398-404. doi: 10.1111/j.1365-277X.2012.01259.x. Epub 2012 May 18.
BACKGROUND: There is no published data describing UK dietary management of urea cycle disorders (UCD). The present study describes dietary practices in UK inherited metabolic disorder (IMD) centres.METHODS: Cross-sectional data from 16 IMD centres were collected by a questionnaire describing the man
PMID 22594780

Japanese Journal

A quantitative study on arginine synthesis from argininosuccinic acid and citrulline by crude enzymes of cattle kidney

SULTANA Halima,INADA Mikiko,WADUD Shaila,TAKAHASHI Toshihiro,MORITA Tetsuo,ONODERA Ryoji
Animal science journal 74(4), 289-294, 2003-08-01
NAID 10018627796

アルギニンパラドックス

中木敏夫,菱川慶一
日本薬理学雑誌 119(1), 7-14, 2002-01-01
L-アルギニン（L-Arg）が尿素回路を構成するアミノ酸であることは古くから知られていたが，今日のように注目を集めるようになったのはNO合成酵素の基質であることが明らかになったからである．NO合成酵素には3種類のアイソザイムがある．3種類のNOSは細胞内L-Arg濃度により，理論的にはすでに十分な基質濃度に達している．L-Argを増やしても活性は最大でもわずかに1%増加するのみであり，差がある反応 …
NAID 130000086738

A Novel Stop Codon Mutation(X465Y) in the Argininosuccinate Lyase Gene in a Patient with Argininosuccinic Aciduria.

, , ,
The Tohoku Journal of Experimental Medicine 198(2), 119-124, 2002
… By mutation analysis of the mRNA and genomic DNA from the patient's leukocytes, we identified a novel missense mutation 1395G>C in the homozygous state, which results in the exchange of a stop codon to tyrosine at amino acid position 465 (X465Y). …
NAID 130004459206

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リンク元	「argininosuccinate」「アルギノコハク酸」
拡張検索	「argininosuccinic aciduria」

「argininosuccinate」

Argininosuccinic acid
Names
	IUPAC name N-[{[(4S)-4-amino-4-carboxybutyl]amino}(imino)methyl]-L-aspartic acid
Identifiers
CAS Number	2387-71-5
ChemSpider	16059
	InChI InChI=1S/C10H18N4O6/c11-5(8(17)18)2-1-3-13-10(12)14-6(9(19)20)4-7(15)16/h5-6H,1-4,11H2,(H,15,16)(H,17,18)(H,19,20)(H3,12,13,14)/t5-,6-/m0/s1 Key: KDZOASGQNOPSCU-WDSKDSINSA-N ; InChI=1/C10H18N4O6/c11-5(8(17)18)2-1-3-13-10(12)14-6(9(19)20)4-7(15)16/h5-6H,1-4,11H2,(H,15,16)(H,17,18)(H,19,20)(H3,12,13,14)/t5-,6-/m0/s1 Key: KDZOASGQNOPSCU-WDSKDSINBJ ;
IUPHAR/BPS	5324
Jmol interactive 3D	Image
PubChem	16950
	SMILES O=C(O)C[C@@H](C(=O)O)NC(=N/CCC[C@H](N)C(=O)O)/N ;
Properties
Chemical formula	C10H18N4O6
Molar mass	290.27312
	Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa).
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	Infobox references