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

2024-04-30 19:32:29 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 3D MOL SDF 3D SDF PDB 3D PDB SMILES InChI

HEADER    PROTEIN                                 05-MAY-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   05-MAY-20         0                                  
HETATM    1  C   UNK     0      -1.341   2.706   0.000  0.00  0.00           C+0
HETATM    2  O   UNK     0      -2.675   1.936   0.000  0.00  0.00           O+0
HETATM    3  C   UNK     0      -1.341  -0.374   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0      -0.007   0.396   0.000  0.00  0.00           C+0
HETATM    5  C   UNK     0      -0.007   1.936   0.000  0.00  0.00           C+0
HETATM    6  N   UNK     0       1.326   2.706   0.000  0.00  0.00           N+0
HETATM    7  O   UNK     0       2.660   0.396   0.000  0.00  0.00           O+0
HETATM    8  C   UNK     0       2.660   1.936   0.000  0.00  0.00           C+0
HETATM    9  N   UNK     0       3.994   2.706   0.000  0.00  0.00           N+0
HETATM   10  C   UNK     0      -1.341  -1.914   0.000  0.00  0.00           C+0
HETATM   11  O   UNK     0      -0.007  -2.684   0.000  0.00  0.00           O+0
HETATM   12  O   UNK     0      -1.341   4.246   0.000  0.00  0.00           O+0
HETATM   13  O   UNK     0      -2.675  -2.684   0.000  0.00  0.00           O+0
CONECT    1    2    5   12                                                  
CONECT    2    1                                                            
CONECT    3    4   10                                                       
CONECT    4    5    3                                                       
CONECT    5    1    4    6                                                  
CONECT    6    8    5                                                       
CONECT    7    8                                                            
CONECT    8    6    9    7                                                  
CONECT    9    8                                                            
CONECT   10    3   11   13                                                  
CONECT   11   10                                                            
CONECT   12    1                                                            
CONECT   13   10                                                            
MASTER        0    0    0    0    0    0    0    0   13    0   24    0
END

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

(2S)-2-(carbamoylamino)pentanedioic acid

Traditional Name

carglumic acid

CAS Registry Number

Not Available

SMILES

NC(=O)N[C@@H](CCC(O)=O)C(O)=O

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

Property	Value	Source
Water Solubility	19.1 g/L	ALOGPS
logP	-1.1	ALOGPS
logP	-1.4	ChemAxon
logS	-1	ALOGPS
pKa (Strongest Acidic)	3.36	ChemAxon
pKa (Strongest Basic)	-2.3	ChemAxon
Physiological Charge	-2	ChemAxon
Hydrogen Acceptor Count	5	ChemAxon
Hydrogen Donor Count	4	ChemAxon
Polar Surface Area	129.72 Å²	ChemAxon
Rotatable Bond Count	5	ChemAxon
Refractivity	39.41 m³·mol⁻¹	ChemAxon
Polarizability	16.79 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Spectra

Spectrum Type	Description	Splash Key	Deposition Date	View
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0a4j-1941000000-35634903a91e28863b1f	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0udi-0910000000-e949449c58d3f3cc2c15	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-000i-9711000000-60f15bbb265b788ea08a	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0fk9-0900000000-5d552fa6b00d42d5e114	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0a4j-1941000000-35634903a91e28863b1f	2018-05-18	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0udi-0910000000-e949449c58d3f3cc2c15	2018-05-18	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-000i-9711000000-60f15bbb265b788ea08a	2018-05-18	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0fk9-0900000000-5d552fa6b00d42d5e114	2018-05-18	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-0006-9500000000-5088c507aa70534ff771	2017-09-01	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positive	splash10-00xr-7491000000-9ae118d744c8a4c43184	2017-10-06	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-000j-0900000000-b40c000081e1b7470cc1	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-002b-0900000000-eb1fe6fcbdc6f376370e	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-0udi-0900000000-245f4056f629592bd99f	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-0udi-4900000000-3a5f21eb40fd1821e24f	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-0pb9-9500000000-67aafe80496b10392ef0	2017-09-14	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-006y-0900000000-764361bc412b5f0ce931	2016-08-03	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-0f6t-3900000000-c2540fd23864f2071f9a	2016-08-03	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-0w2m-9300000000-11ad8cdea4f07b49e70a	2016-08-03	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-0005-3900000000-1b6012a21fc5e4f08ab1	2016-08-03	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-0f6w-6900000000-2f0bc6673aa76c508051	2016-08-03	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-0006-9100000000-ad1bb50857e6e68f1810	2016-08-03	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-0002-0900000000-d6bc8a160d559706e590	2021-10-11	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-0f89-2900000000-e78d6309a9f45260eafb	2021-10-11	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-000l-9000000000-d0b7e0dec04859c09147	2021-10-11	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-004i-0900000000-d986898d35880221c670	2021-10-11	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-0ufr-0900000000-0e99b13b20a88bbcd9ec	2021-10-11	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-0udl-4900000000-a30b3ee9c9bc68a482d0	2021-10-11	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-16	View Spectrum

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	Source Sub-type	Species	Data Source	Reference

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:0x00007fd0f935ff08>

3D MOL for #<Metabolite:0x00007fd0f935ff08>

3D SDF for #<Metabolite:0x00007fd0f935ff08>

3D-SDF for #<Metabolite:0x00007fd0f935ff08>

PDB for #<Metabolite:0x00007fd0f935ff08>

3D PDB for #<Metabolite:0x00007fd0f935ff08>

SMILES for #<Metabolite:0x00007fd0f935ff08>

INCHI for #<Metabolite:0x00007fd0f935ff08>

Structure for #<Metabolite:0x00007fd0f935ff08>

3D Structure for #<Metabolite:0x00007fd0f935ff08>