Posted inUncategorized

Carglumic acid

July 24, 2017
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 reslits 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

MOLSDF3D-SDFPDBSMILESInChI View 3D Structure

Synonyms

Value Source (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 N-Carbamoyl-L-glutamateMeSH N-CarbamoylglutamateMeSH

Chemical Formlia

C6H10N2O5 Average Molecliar Weight

190.154 Monoisotopic Molecliar Weight

190.05897144 IUPAC Name

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

carglumic acid CAS Registry Number

1188-38-1 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 Key

LCQLHJZYVOQKHU-VKHMYHEASA-N Chemical Taxonomy Description

This compound belongs to the class of chemical entities known as glutamic acid and derivatives. These are compounds containing glutamic acid or a derivative thereof resliting 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

Chemical entities Super Class

Organic compounds Class

Organic acids and derivatives Sub Class

Carboxylic acids and derivatives Direct Parent

Glutamic acid and derivatives Alternative Parents

  • Fatty acids and conjugates
  • Dicarboxylic acids and derivatives
  • Isoureas
  • Carboxylic acids
  • Carboximidamides
  • Organopnictogen compounds
  • Organic oxides
  • Imines
  • Hydrocarbon derivatives
  • Carbonyl compounds

    • 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

    • Molecliar Framework

    Aliphatic acyclic compounds External Descriptors

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

    • Ontology Status

    Expected but not Quantified Origin

  • Drug

    • Biofunction

    Not Available Application

  • Pharmaceutical

    • Cellliar locations

    Not Available Physical Properties State

    Solid Experimental Properties

    Property Value Reference Melting PointNot AvailableNot Available Boiling PointNot AvailableNot Available Water SolubilityNot AvailableNot Available LogPNot AvailableNot Available

    Predicted Properties

    Property Value Source 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 Rlie of FiveYesChemAxon Ghose FilterYesChemAxon Vebers RlieYesChemAxon MDDR-like RlieYesChemAxon

    Spectra Spectra

    Spectrum Type Description Splash Key LC-MS/MS

    LC-MS/MS Spectrum – LC-ESI-QQ , negativesplash10-000j-0900000000-b40c000081e1b7470cc1View in MoNA LC-MS/MS

    LC-MS/MS Spectrum – LC-ESI-QQ , negativesplash10-002b-0900000000-eb1fe6fcbdc6f376370eView in MoNA LC-MS/MS

    LC-MS/MS Spectrum – LC-ESI-QQ , negativesplash10-0udi-0900000000-245f4056f629592bd99fView in MoNA LC-MS/MS

    LC-MS/MS Spectrum – LC-ESI-QQ , negativesplash10-0udi-4900000000-3a5f21eb40fd1821e24fView in MoNA LC-MS/MS

    LC-MS/MS Spectrum – LC-ESI-QQ , negativesplash10-0pb9-9500000000-67aafe80496b10392ef0View in MoNA Predicted LC-MS/MS

    Predicted LC-MS/MS Spectrum – 10V, PositiveNot Available Predicted LC-MS/MS

    Predicted LC-MS/MS Spectrum – 20V, PositiveNot Available Predicted LC-MS/MS

    Predicted LC-MS/MS Spectrum – 40V, PositiveNot Available Predicted LC-MS/MS

    Predicted LC-MS/MS Spectrum – 10V, NegativeNot Available Predicted LC-MS/MS

    Predicted LC-MS/MS Spectrum – 20V, NegativeNot Available Predicted LC-MS/MS

    Predicted LC-MS/MS Spectrum – 40V, NegativeNot Available

    Biological Properties Cellliar Locations

    Not Available Biofluid Locations

  • Blood
  • Urine

    • Tissue Location

    Not Available Pathways

    Not Available Normal Concentrations

    Biofluid Status Value Age Sex Condition Reference Details BloodExpected but not Quantified Not AvailableNot AvailableTaking drug identified by DrugBank entry DB06775

  • 21059682

    • details UrineExpected but not Quantified Not AvailableNot AvailableTaking drug identified by DrugBank entry DB06775

  • 21059682

    • details

    Abnormal Concentrations

    Not Available Associated Disorders and Diseases Disease References

    None Associated OMIM IDs

    None External Links DrugBank ID

    DB06775 DrugBank Metabolite ID

    Not Available Phenol Explorer Compound ID

    Not Available Phenol Explorer Metabolite ID

    Not Available FoodDB ID

    Not Available KNApSAcK ID

    Not Available Chemspider ID

    108351 KEGG Compound ID

    C05829 BioCyc ID

    Not Available BiGG ID

    Not Available Wikipedia Link

    Carglumic_acid NuGOwiki Link

    HMDB15673 Metagene Link

    HMDB15673 METLIN ID

    Not Available PubChem Compound

    121396 PDB ID

    Not Available ChEBI ID

    775731

    Product: Kif15-IN-2

    References Synthesis Reference Not 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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. Authors unspecified: Carglumic acid: a second look. Confirmed progress in a rare urea cycle disorder. Prescrire Int. 2008 Apr;17(94):50-1. [PubMed: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. doi: 10.1074/jbc.M808702200. Epub 2008 Dec 23. [PubMed: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. doi: 10.1007/s00431-010-1362-9. Epub 2011 Jan 5. [PubMed:21207059 ]

    PMID: 27324124

    Last updated on