You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Record Information
Version3.6
Creation Date2012-09-06 15:16:52 UTC
Update Date2016-02-11 01:33:56 UTC
HMDB IDHMDB15673
Secondary Accession NumbersNone
Metabolite Identification
Common NameCarglumic acid
DescriptionCarglumic 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
Thumb
Synonyms
ValueSource
(2S)-2-(carbamoylamino)Pentanedioic acidChEBI
Acide carglumiqueChEBI
acido CarglumicoChEBI
Acidum carglumicumChEBI
CarbagluChEBI
carbamino-L-Glutamic acidChEBI
Carbamylglutamic acidChEBI
L-N-Carbamoylglutamic acidChEBI
Ureidoglutaric acidChEBI
(2S)-2-(carbamoylamino)PentanedioateGenerator
CarglumateGenerator
carbamino-L-GlutamateGenerator
CarbamylglutamateGenerator
L-N-CarbamoylglutamateGenerator
UreidoglutarateGenerator
(S)-2-Ureidopentanedioic acidHMDB
N-Carbamoyl-L-glutamic acidHMDB
N-Carbamyl-L-glutamateHMDB
N-CarbamylglutamateHMDB
Chemical FormulaC6H10N2O5
Average Molecular Weight190.154
Monoisotopic Molecular Weight190.05897144
IUPAC Name(2S)-2-(carbamoylamino)pentanedioic acid
Traditional Namecarglumic acid
CAS Registry Number1188-38-1
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 KeyInChIKey=LCQLHJZYVOQKHU-VKHMYHEASA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as n-carbamoyl-alpha amino acids. These are compounds containing an alpha amino acid which bears an carbamoyl group at its terminal nitrogen atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentN-carbamoyl-alpha amino acids
Alternative Parents
Substituents
  • N-carbamoyl-alpha-amino acid
  • Amino fatty acid
  • Fatty acyl
  • Fatty acid
  • Dicarboxylic acid or derivatives
  • Urea
  • Carboxylic acid
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
StatusExpected but not Quantified
Origin
  • Drug
BiofunctionNot Available
Application
  • Pharmaceutical
Cellular locationsNot Available
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility19.1 mg/mLALOGPS
logP-1.1ALOGPS
logP-1.4ChemAxon
logS-1ALOGPS
pKa (Strongest Acidic)3.36ChemAxon
pKa (Strongest Basic)-2.3ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count4ChemAxon
Polar Surface Area129.72 Å2ChemAxon
Rotatable Bond Count5ChemAxon
Refractivity39.41 m3·mol-1ChemAxon
Polarizability16.78 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, NegativeNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, NegativeNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, NegativeNot Available
Biological Properties
Cellular LocationsNot Available
Biofluid Locations
  • Blood
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodExpected but not QuantifiedNot ApplicableNot AvailableNot AvailableTaking drug identified by DrugBank entry DB06775
  • Not Applicable
details
UrineExpected but not QuantifiedNot ApplicableNot AvailableNot AvailableTaking drug identified by DrugBank entry DB06775
  • Not Applicable
details
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDDB06775
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDNot Available
KNApSAcK IDNot Available
Chemspider ID108351
KEGG Compound IDC05829
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkCarglumic_acid
NuGOwiki LinkHMDB15673
Metagene LinkHMDB15673
METLIN IDNot Available
PubChem Compound121396
PDB IDNot Available
ChEBI ID775731
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. 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. [12447942 ]
  2. 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. [15480384 ]
  3. Thompson CA: Carglumic acid approved to treat genetic hyperammonemia. Am J Health Syst Pharm. 2010 May 1;67(9):690. doi: 10.2146/news100031. [20410539 ]
  4. 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. [21941437 ]
  5. European Medicines Agency [Link]

Enzymes

General function:
Involved in catalytic activity
Specific function:
Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.
Gene Name:
CPS1
Uniprot ID:
P31327
Molecular weight:
165649.075
References
  1. [No authors listed]Carglumic acid: a second look. Confirmed progress in a rare urea cycle disorder. Prescrire Int. 2008 Apr;17(94):50-1. [18516804 ]
  2. Hart EJ, Powers-Lee SG: Role of Cys-1327 and Cys-1337 in redox sensitivity and allosteric monitoring in human carbamoyl phosphate synthetase. J Biol Chem. 2009 Feb 27;284(9):5977-85. Epub 2008 Dec 23. [19106093 ]
  3. Kasapkara CS, Ezgu FS, Okur I, Tumer L, Biberoglu G, Hasanoglu A: N-carbamylglutamate treatment for acute neonatal hyperammonemia in isovaleric acidemia. Eur J Pediatr. 2011 Jun;170(6):799-801. Epub 2011 Jan 5. [21207059 ]