MiMeDB: Showing metabocard for (N(omega)-L-arginino)succinic acid (MMDBc0033045)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2021-11-19 04:32:22 UTC

Update Date

2022-12-15 22:51:53 UTC

MiMeDB ID

MMDBc0033045

Metabolite Identification

Common Name

(N(omega)-L-arginino)succinic acid

Description

Structure

MOL SDF PDB SMILES InChI

Synonyms

Not Available

Chemical Formula

C₁₀H₁₈N₄O₆

Average Molecular Weight

290.2731

Monoisotopic Molecular Weight

290.122634328

IUPAC Name

Not Available

Traditional Name

Not Available

CAS Registry Number

2387-71-5

SMILES

Not Available

InChI Identifier

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

InChI Key

KDZOASGQNOPSCU-ZBHICJROSA-N

Chemical Taxonomy

Functional Ontology

Not Available

Physical Properties

State

Solid

Predicted Properties

Not Available

Spectra

Not Available

Biological Properties

Cellular Locations

Cytoplasm

Biospecimen Locations

Blood
Kidney
Placenta
Prostate
Urine

Tissue Locations

Not Available

Associated OMIM IDs

114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)

Human Proteins and Enzymes

Proteins

Human Pathways

Pathways

Name	SMPDB/PathBank

Metabolic Reactions

Not Available

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Related Health Condition	Evidence Type	Measured in Matrix	Reference
Argininosuccinic aciduria	Increased	This associated disease is originally imported from HMDB metabolite HMDB0000052 concentration id 136708	Association	Human Urine	12408190	details
Argininosuccinic aciduria	Increased	This associated disease is originally imported from HMDB metabolite HMDB0000052 concentration id 136707	Association	Human Blood	12408190	details
Argininosuccinic aciduria	Increased	This associated disease is originally imported from HMDB metabolite HMDB0000052 concentration id 59280	Association	Human Urine	5075233	details
Argininemia		This associated disease is originally imported from HMDB metabolite HMDB0000052 concentration id 58909	Association	Human Urine	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)	details

Microbial Sources

Kingdom	Phylum	Total species	Hosts and Body Sites	Microbial Links
Fungi	Ascomycota	1	Human Feces	Unknown

Exposure Sources

Source Type	Source Sub-type	Species	Data Source	Reference	Details

Host Biospecimen and Location

Host and Biospecimen	Status	Mean	Min	Max	Age	Sex	Health Condition	Data Source	Reference
Human	Detected and Quantified	667			Infant (0-1 year old)	Male	Argininosuccinic aciduria (ASL)	HMDB	12408190	details
Human	Detected and Quantified	1515			Infant (0-1 year old)	Male	Argininosuccinic aciduria (ASL)	HMDB	12408190	details
Human	Detected and Quantified	1542			Adult (>18 years old)	Both	Argininosuccinic aciduria (ASL)	HMDB	5075233	details
Human	Detected and Quantified	300.0	270.0	458.0	Children (1-13 years old)	Female	Argininosuccinyl-CoA lyase deficiency	HMDB	8582405	details
Human	Detected and Quantified	0			Adult (>18 years old)	Both	Normal	HMDB	21312326	details
Human	Detected and Quantified	<2			Infant (0-1 year old)	Male	Normal	HMDB	12408190	details
Human	Detected and Quantified	<4			Infant (0-1 year old)	Male	Normal	HMDB	12408190	details
Human	Detected and Quantified		0	99	Newborn (0-30 days old)	Both	Normal	HMDB	31506554	details
Human	Detected and Quantified	0.65	0.0	1.3	Children (1-13 years old)	Both	Argininemia	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)	details
Human	Detected and Quantified		0	1	Children (1 - 13 years old)	Both	Normal	HMDB	Argininosuccinate Lyase Deficiency. 2011 Feb 3 [Updated 2012 Feb 2]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. Available from: https://www.ncbi.nlm.nih.gov/books/NBK51784/GeneReviews®. Seattle (WA).	details
Human	Detected and Quantified		0	5	Newborn (0-30 days old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab	details
Human	Detected and Quantified		0	5	Infant (1 - 3 months old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab	details
Human	Detected and Quantified		0	5	Children (3 months - 6 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab	details
Human	Detected and Quantified		0	5	Children (6 - 18 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab	details
Human	Detected and Quantified	<0.433			Not Specified	Not Specified	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.	details
Human	Detected and Quantified	<1.70			Adult (>18 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)	details
Human	Detected and Quantified	<2.71			Children (1-13 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)	details
Human	Detected and Quantified	<4.19			Children (1-13 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)	details
Human	Detected and Quantified	<5.43			Children (1-13 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)	details
Human	Detected and Quantified	<7.81			Adolescent (13-18 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)	details
Human	Detected and Quantified	<8.71			Infant (0-1 year old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)	details
Human Kidney	Detected but not Quantified				Not Specified	Not Specified	Normal	HMDB	34986597	details
Human Placenta	Detected but not Quantified				Not Specified	Not Specified	Normal	HMDB	34986597	details
Human Prostate	Detected but not Quantified				Not Specified	Not Specified	Normal	HMDB	34986597	details

External Links

Not Available

References

Synthesis Reference

Not Available

General References

Glick NR, Snodgrass PJ, Schafer IA: Neonatal argininosuccinic aciduria with normal brain and kidney but absent liver argininosuccinate lyase activity. Am J Hum Genet. 1976 Jan;28(1):22-30. [PubMed:174426 ]
Fleisher LD, Rassin DK, Desnick RJ, Salwen HR, Rogers P, Bean M, Gaull GE: Argininosuccinic aciduria: prenatal studies in a family at risk. Am J Hum Genet. 1979 Jul;31(4):439-45. [PubMed:484552 ]
Michels VV, Beaudet AL: Arginase deficiency in multiple tissues in argininemia. Clin Genet. 1978 Jan;13(1):61-7. doi: 10.1111/j.1399-0004.1978.tb04128.x. [PubMed:624188 ]
Pelli N, Fensom AH, Slade C, Boa F, Mieli-Vergani G, Vergani D: Argininosuccinate lyase: a new autoantigen in liver disease. Clin Exp Immunol. 1998 Dec;114(3):455-61. doi: 10.1046/j.1365-2249.1998.00754.x. [PubMed:9844057 ]
Wasant P, Srisomsap C, Liammongkolkul S, Svasti J: Urea cycle disorders in Thai infants: a report of 5 cases. J Med Assoc Thai. 2002 Aug;85 Suppl 2:S720-31. [PubMed:12403252 ]
Kleijer WJ, Garritsen VH, Linnebank M, Mooyer P, Huijmans JG, Mustonen A, Simola KO, Arslan-Kirchner M, Battini R, Briones P, Cardo E, Mandel H, Tschiedel E, Wanders RJ, Koch HG: Clinical, enzymatic, and molecular genetic characterization of a biochemical variant type of argininosuccinic aciduria: prenatal and postnatal diagnosis in five unrelated families. J Inherit Metab Dis. 2002 Sep;25(5):399-410. doi: 10.1023/a:1020108002877. [PubMed:12408190 ]
Reid Sutton V, Pan Y, Davis EC, Craigen WJ: A mouse model of argininosuccinic aciduria: biochemical characterization. Mol Genet Metab. 2003 Jan;78(1):11-6. doi: 10.1016/s1096-7192(02)00206-8. [PubMed:12559843 ]
Au WL, Lim TC, Seow DC, Koh PL, Loh NK, Lim MS, Tan IK, Yee WC: Serial diffusion-weighted magnetic resonance imaging in adult-onset citrullinaemia. J Neurol Sci. 2003 May 15;209(1-2):101-4. doi: 10.1016/s0022-510x(02)00439-2. [PubMed:12686410 ]
Cohen BD: Methyl group deficiency and guanidino production in uremia. Mol Cell Biochem. 2003 Feb;244(1-2):31-6. [PubMed:12701806 ]
Scaglia F, Brunetti-Pierri N, Kleppe S, Marini J, Carter S, Garlick P, Jahoor F, O'Brien W, Lee B: Clinical consequences of urea cycle enzyme deficiencies and potential links to arginine and nitric oxide metabolism. J Nutr. 2004 Oct;134(10 Suppl):2775S-2782S; discussion 2796S-2797S. doi: 10.1093/jn/134.10.2775S. [PubMed:15465784 ]
Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

Showing metabocard for (N(omega)-L-arginino)succinic acid (MMDBc0033045)

MOL for #<Metabolite:0x000055b76df3d458>

3D MOL for #<Metabolite:0x000055b76df3d458>

3D SDF for #<Metabolite:0x000055b76df3d458>

3D-SDF for #<Metabolite:0x000055b76df3d458>

PDB for #<Metabolite:0x000055b76df3d458>

3D PDB for #<Metabolite:0x000055b76df3d458>

SMILES for #<Metabolite:0x000055b76df3d458>

INCHI for #<Metabolite:0x000055b76df3d458>

Structure for #<Metabolite:0x000055b76df3d458>

3D Structure for #<Metabolite:0x000055b76df3d458>