MiMeDB: Showing metabocard for Carglumic acid (MMDBc0000465)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2020-12-10 18:38:23 UTC

Update Date

2024-10-11 03:42:11 UTC

Metabolite ID

MMDBc0000465

Metabolite Identification

Common Name

Carglumic acid

Description

Carglumic acid is an orphan drug used for the treatment of hyperammonaemia in patients with N-acetylglutamate synthase deficiency. This rare genetic disorder results in elevated blood levels of ammonia, which can eventually cross the blood-brain barrier and cause neurologic problems, cerebral edema, coma, and death. Carglumic acid was approved by the U.S. Food and Drug Administration (FDA) on 18 March 2010.

Structure

MOL SDF PDB SMILES InChI

Synonyms

Value	Source
(2S)-2-(Carbamoylamino)pentanedioic acid	ChEBI
Acide carglumique	ChEBI
Acido carglumico	ChEBI
Acidum carglumicum	ChEBI
Carbaglu	ChEBI
Carbamino-L-glutamic acid	ChEBI
Carbamylglutamic acid	ChEBI
L-N-Carbamoylglutamic acid	ChEBI
Ureidoglutaric acid	ChEBI
(2S)-2-(Carbamoylamino)pentanedioate	Generator
Carbamino-L-glutamate	Generator
Carbamylglutamate	Generator
L-N-Carbamoylglutamate	Generator
Ureidoglutarate	Generator
Carglumate	Generator
(S)-2-Ureidopentanedioic acid	HMDB
N-Carbamoyl-L-glutamic acid	HMDB
N-Carbamyl-L-glutamate	HMDB
N-Carbamylglutamate	HMDB
N-Carbamoyl-L-glutamate	HMDB
N-Carbamoylglutamate	HMDB

Molecular Formula

C₆H₁₀N₂O₅

Average Mass

190.154

Monoisotopic Mass

190.05897144

IUPAC Name

Not Available

Traditional Name

Not Available

CAS Registry Number

Not Available

SMILES

Not Available

InChI Identifier

InChI=1S/C6H10N2O5/c7-6(13)8-3(5(11)12)1-2-4(9)10/h3H,1-2H2,(H,9,10)(H,11,12)(H3,7,8,13)/t3-/m0/s1

InChI Key

LCQLHJZYVOQKHU-VKHMYHEASA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as glutamic acid and derivatives. Glutamic acid and derivatives are compounds containing glutamic acid or a derivative thereof resulting from reaction of glutamic acid at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Amino acids, peptides, and analogues

Direct Parent

Glutamic acid and derivatives

Alternative Parents

Substituents

Glutamic acid or derivatives
Dicarboxylic acid or derivatives
Fatty acid
Isourea
Carboximidamide
Carboxylic acid
Carboximidic acid derivative
Organic oxide
Organic nitrogen compound
Hydrocarbon derivative
Carbonyl group
Organooxygen compound
Organonitrogen compound
Imine
Organopnictogen compound
Organic oxygen compound
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

ureas (CHEBI:71028 )
N-acyl-L-glutamic acid (CHEBI:71028 )

Functional Ontology

Not Available

Physical Properties

State

Expected Solid

Predicted Properties

Not Available

Spectra

Not Available

Biological Properties

Cellular Locations

Not Available

Biospecimen Locations

Feces

Tissue Locations

Not Available

Associated OMIM IDs

114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)

Human Proteins and Enzymes

Protein ID	Protein Name	Protein Type	Uniprot ID	Species
MMDBp0192027	Carbamoyl-phosphate synthase [ammonia], mitochondrial	Enzyme	P31327	Homo sapiens

Human Pathways

Pathways

Name	SMPDB/PathBank

Metabolic Reactions

Not Available

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Evidence Type	Host and Biospecimen	Data Source	Reference

Microbial Sources

Superkingdom/Kingdom	Phylum	Total species	Hosts and Body Sites	Microbial Links
Bacteria	Firmicutes	2	Human ; Human feces; Human stool	Indirect association between microbiota composition and metabolites measured in host biospecimen
Bacteria	Bacteroidetes	1	Human feces; Human stool	Indirect association between microbiota composition and metabolites measured in host biospecimen

Exposure Sources

Source Type	Activity	Data Source	Reference
Drugs	Not Available	U.S. Environmental Protection Agency	CompTox
Drugs	Antimicrobial	U.S. Environmental Protection Agency	CompTox
Cosmetics	Not Available	U.S. Environmental Protection Agency	CompTox
Domestics	Not Available	U.S. Environmental Protection Agency	CompTox

Host Biospecimen and Location

Host and Biospecimen	Status	Mean	Std	Min	Max	Units	Age	Sex	Health Condition	Data Source	Reference
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	29808030

External Links

HMDB ID

HMDB0015673

DrugBank ID

DB06775

Phenol Explorer Compound ID

Not Available

FooDB ID

Not Available

KNApSAcK ID

Not Available

Chemspider ID

108351

KEGG Compound ID

C05829

BioCyc ID

N-CARBAMYL-L-GLUTAMATE

BiGG ID

Not Available

Wikipedia Link

Carglumic_acid

METLIN ID

Not Available

PubChem Compound

121396

PDB ID

Not Available

ChEBI ID

71028

Food Biomarker Ontology

Not Available

References

Synthesis Reference

Not Available

General References

Elpeleg O, Shaag A, Ben-Shalom E, Schmid T, Bachmann C: N-acetylglutamate synthase deficiency and the treatment of hyperammonemic encephalopathy. Ann Neurol. 2002 Dec;52(6):845-9. doi: 10.1002/ana.10406. [PubMed:12447942 ]
Caldovic L, Morizono H, Daikhin Y, Nissim I, McCarter RJ, Yudkoff M, Tuchman M: Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate. J Pediatr. 2004 Oct;145(4):552-4. doi: 10.1016/j.jpeds.2004.06.047. [PubMed:15480384 ]
Thompson CA: Carglumic acid approved to treat genetic hyperammonemia. Am J Health Syst Pharm. 2010 May 1;67(9):690. doi: 10.2146/news100031. [PubMed:20410539 ]
Haberle J: Role of carglumic acid in the treatment of acute hyperammonemia due to N-acetylglutamate synthase deficiency. Ther Clin Risk Manag. 2011;7:327-32. doi: 10.2147/TCRM.S12703. Epub 2011 Aug 2. [PubMed:21941437 ]
European Medicines Agency [Link]

Showing metabocard for Carglumic acid (MMDBc0000465)

MOL for #<Metabolite:0x00007fdb0dfb1fb8>

3D MOL for #<Metabolite:0x00007fdb0dfb1fb8>

3D SDF for #<Metabolite:0x00007fdb0dfb1fb8>

3D-SDF for #<Metabolite:0x00007fdb0dfb1fb8>

PDB for #<Metabolite:0x00007fdb0dfb1fb8>

3D PDB for #<Metabolite:0x00007fdb0dfb1fb8>

SMILES for #<Metabolite:0x00007fdb0dfb1fb8>

INCHI for #<Metabolite:0x00007fdb0dfb1fb8>

Structure for #<Metabolite:0x00007fdb0dfb1fb8>

3D Structure for #<Metabolite:0x00007fdb0dfb1fb8>