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Record Information
Version1.0
Created at2005-11-16 15:48:42 UTC
Updated at2020-11-24 22:13:29 UTC
NP-MRD IDNP0000182
Secondary Accession NumbersNone
Natural Product Identification
Common NameHomocarnosine
DescriptionHomocarnosine is a normal human metabolite, the brain-specific dipeptide of gamma-aminobutyric acid (GABA) and histidine. (PMID 1266573 ). Increased concentration of CSF homocarnosine has been found in familial spastic paraplegia. (PMID 842287 ). Homocarnosinosis (an inherited disorder, OMIM 236130 ) is characterized by an elevated level of the dipeptide homocarnosine (Hca) in the Cerebrospinal fluid (CSF) and the brain and by carnosinuria and serum carnosinase deficiency, and can co-exist with paraplegia, retinitis pigmentosa, and a progressive mental deficiency. (PMID 3736769 ). In glial tumors of human brain the content of homocarnosine has been found to be lower than in brain tissue (PMID 1032224 ), while an increase in content of homocarnosine was observed in brain tissue of animals under experimental trauma of cranium. (PMID 1025883 ).
Structure
Thumb
Synonyms
ValueSource
(2S)-HomocarnosineChEBI
(S)-HomocarnosineChEBI
gamma-Aminobutyryl-L-hisChEBI
N-(4-Aminobutanoyl)-(S)-histidineChEBI
N-(4-Aminobutyryl)-L-histidineChEBI
N(alpha)-(4-Aminobutyryl)-L-histidineChEBI
L-HomocarnosineKegg
gamma-Aminobutyryl histidineKegg
N-(4-Amino-1-oxobutyryl)histidineKegg
g-Aminobutyryl-L-hisGenerator
Γ-aminobutyryl-L-hisGenerator
N(a)-(4-Aminobutyryl)-L-histidineGenerator
N(Α)-(4-aminobutyryl)-L-histidineGenerator
g-Aminobutyryl histidineGenerator
Γ-aminobutyryl histidineGenerator
gamma-Aminobutyryl-L-histidineHMDB
Homocarnosine sulfateHMDB
g-Aminobutyryl-L-histidineHMDB
g-AminobutyrylhistidineHMDB
gamma-AminobutyrylhistidineHMDB
N-(4-Aminobutyryl)-histidineHMDB
Chemical FormulaC10H16N4O3
Average Mass240.2590 Da
Monoisotopic Mass240.12224 Da
IUPAC Name(2S)-2-(4-aminobutanamido)-3-(1H-imidazol-4-yl)propanoic acid
Traditional Name(2S)-2-(4-aminobutanamido)-3-(1H-imidazol-4-yl)propanoic acid
CAS Registry Number3650-73-5
SMILES
NCCCC(=O)N[C@@H](CC1=CNC=N1)C(O)=O
InChI Identifier
InChI=1S/C10H16N4O3/c11-3-1-2-9(15)14-8(10(16)17)4-7-5-12-6-13-7/h5-6,8H,1-4,11H2,(H,12,13)(H,14,15)(H,16,17)/t8-/m0/s1
InChI KeyCCLQKVKJOGVQLU-QMMMGPOBSA-N
Spectra
Spectrum TypeDescriptionDepositor IDDeposition DateView
1D NMR1H NMR Spectrum (1D, 500 MHz, H2O, experimental)Wishart Lab2021-06-20View Spectrum
2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)Wishart Lab2021-06-20View Spectrum
Species
Species of Origin
  • Eubacteria
  • Chemical Taxonomy
    Description Belongs to the class of organic compounds known as hybrid peptides. Hybrid peptides are compounds containing at least two different types of amino acids (alpha, beta, gamma, delta) linked to each other through a peptide bond.
    KingdomOrganic compounds
    Super ClassOrganic acids and derivatives
    ClassPeptidomimetics
    Sub ClassHybrid peptides
    Direct ParentHybrid peptides
    Alternative Parents
    Substituents
    • Hybrid peptide
    • Histidine or derivatives
    • N-acyl-alpha-amino acid
    • N-acyl-alpha amino acid or derivatives
    • N-acyl-l-alpha-amino acid
    • Gamma amino acid or derivatives
    • Alpha-amino acid or derivatives
    • Imidazolyl carboxylic acid derivative
    • N-acyl-amine
    • Fatty amide
    • Fatty acyl
    • Azole
    • Imidazole
    • Heteroaromatic compound
    • Amino acid
    • Amino acid or derivatives
    • Secondary carboxylic acid amide
    • Carboxamide group
    • Carboxylic acid derivative
    • Carboxylic acid
    • Azacycle
    • Organoheterocyclic compound
    • Monocarboxylic acid or derivatives
    • Organic oxygen compound
    • Hydrocarbon derivative
    • Amine
    • Organic nitrogen compound
    • Organic oxide
    • Carbonyl group
    • Primary amine
    • Organooxygen compound
    • Primary aliphatic amine
    • Organopnictogen compound
    • Organonitrogen compound
    • Aromatic heteromonocyclic compound
    Molecular FrameworkAromatic heteromonocyclic compounds
    External Descriptors
    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 Solubility7.37 g/LALOGPS
    logP-2.8ALOGPS
    logP-3.9ChemAxon
    logS-1.5ALOGPS
    pKa (Strongest Acidic)3.45ChemAxon
    pKa (Strongest Basic)9.99ChemAxon
    Physiological Charge0ChemAxon
    Hydrogen Acceptor Count5ChemAxon
    Hydrogen Donor Count4ChemAxon
    Polar Surface Area121.1 ŲChemAxon
    Rotatable Bond Count7ChemAxon
    Refractivity59.75 m³·mol⁻¹ChemAxon
    Polarizability24.49 ųChemAxon
    Number of Rings1ChemAxon
    BioavailabilityYesChemAxon
    Rule of FiveYesChemAxon
    Ghose FilterNoChemAxon
    Veber's RuleNoChemAxon
    MDDR-like RuleNoChemAxon
    HMDB IDHMDB0000745
    DrugBank IDNot Available
    Phenol Explorer Compound IDNot Available
    FoodDB IDFDB022217
    KNApSAcK IDNot Available
    Chemspider ID8418848
    KEGG Compound IDC00884
    BioCyc IDCPD-12185
    BiGG IDNot Available
    Wikipedia LinkNot Available
    METLIN ID270
    PubChem Compound10243361
    PDB IDNot Available
    ChEBI ID85981
    Good Scents IDNot Available
    References
    General References
    1. Lenney JF, Peppers SC, Kucera CM, Sjaastad O: Homocarnosinosis: lack of serum carnosinase is the defect probably responsible for elevated brain and CSF homocarnosine. Clin Chim Acta. 1983 Aug 15;132(2):157-65. [PubMed:6616870 ]
    2. 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 ]
    3. Petroff OA, Mattson RH, Behar KL, Hyder F, Rothman DL: Vigabatrin increases human brain homocarnosine and improves seizure control. Ann Neurol. 1998 Dec;44(6):948-52. [PubMed:9851440 ]
    4. Petroff OA, Hyder F, Mattson RH, Rothman DL: Topiramate increases brain GABA, homocarnosine, and pyrrolidinone in patients with epilepsy. Neurology. 1999 Feb;52(3):473-8. [PubMed:10025774 ]
    5. Rothman DL, Behar KL, Prichard JW, Petroff OA: Homocarnosine and the measurement of neuronal pH in patients with epilepsy. Magn Reson Med. 1997 Dec;38(6):924-9. [PubMed:9402193 ]
    6. Pearl PL, Novotny EJ, Acosta MT, Jakobs C, Gibson KM: Succinic semialdehyde dehydrogenase deficiency in children and adults. Ann Neurol. 2003;54 Suppl 6:S73-80. [PubMed:12891657 ]
    7. Tell G, Bohlen P, Schechter PJ, Koch-Weser J, Agid Y, Bonnet AM, Coquillat G, Chazot G, Fischer C: Treatment of Huntington disease with gamma-acetylenic GABA an irreversible inhibitor of GABA-transaminase: increased CSF GABA and homocarnosine without clinical amelioration. Neurology. 1981 Feb;31(2):207-11. [PubMed:6258106 ]
    8. Promyslov MSh, Mirzoian RA: [Homocarnosine metabolism in brain tissue]. Vopr Biokhim Mozga. 1976;11:71-7. [PubMed:1032224 ]
    9. Iurovskii SL, Orlov VI, Pogorelova TN, Drukker NA, Madan M: [Homocarnosine of the amniotic fluid as an indicator of fetal cerebral lesions]. Akush Ginekol (Mosk). 1989 Sep;(9):45-7. [PubMed:2596637 ]
    10. Menachem EB, Persson LI, Schechter PJ, Haegele KD, Huebert N, Hardenberg J, Dahlgren L, Mumford JP: Effects of single doses of vigabatrin on CSF concentrations of GABA, homocarnosine, homovanillic acid and 5-hydroxyindoleacetic acid in patients with complex partial epilepsy. Epilepsy Res. 1988 Mar-Apr;2(2):96-101. [PubMed:3143561 ]
    11. Perry TL, Kish SJ, Sjaastad O, Gjessing LR, Nesbakken R, Schrader H, Loken AC: Homocarnosinosis: increased content of homocarnosine and deficiency of homocarnosinase in brain. J Neurochem. 1979 Jun;32(6):1637-40. [PubMed:448356 ]
    12. Promyslov MSh, Mirzoian RA: [Study of homocarnosine metabolism in the brain tissue in diseases of the nervous system]. Vopr Med Khim. 1976 Mar-Apr;22(2):209-12. [PubMed:1025883 ]
    13. Manyam BV, Bhatt MH, Moore WD, Devleschoward AB, Anderson DR, Calne DB: Bilateral striopallidodentate calcinosis: cerebrospinal fluid, imaging, and electrophysiological studies. Ann Neurol. 1992 Apr;31(4):379-84. [PubMed:1586138 ]
    14. Sjaastad O, Gjessing L, Berstad JR, Gjesdahl P: Homocarnosinosis. 3. Spinal fluid amino acids in familial spastic paraplegia. Acta Neurol Scand. 1977 Feb;55(2):158-62. [PubMed:842287 ]
    15. Sjaastad O, Berstad J, Gjesdahl P, Gjessing L: Homocarnosinosis. 2. A familial metabolic disorder associated with spastic paraplegia, progressive mental deficiency, and retinal pigmentation. Acta Neurol Scand. 1976 Apr;53(4):275-90. [PubMed:1266573 ]
    16. Lunde HA, Gjessing LR, Sjaastad O: Homocarnosinosis: influence of dietary restriction of histidine. Neurochem Res. 1986 Jun;11(6):825-38. [PubMed:3736769 ]