Record Information
Version 1.0
Update Date 1/22/2018 11:54:54 AM
Metabolite IDPAMDB000182
Identification
Name: Homocarnosine
Description:Homocarnosine is an intermediate of arginine and proline metabolism. tis converted from 4-amino-butanoate via aminoacylhistidine dipeptidase. It consists of gamma-aminobutyric acid and histidine.
Structure
Thumb
Synonyms:
  • G-Aminobutyryl-L-histidine
  • G-Aminobutyrylhistidine
  • Gamma-Aminobutyryl-L-histidine
  • Gamma-Aminobutyrylhistidine
  • Homocarnosine
  • L-Homocarnosine
  • N-(4-Aminobutyryl)-Histidine
  • N-(4-Aminobutyryl)-L-Histidine
  • γ-Aminobutyryl-L-histidine
  • γ-Aminobutyrylhistidine
Chemical Formula: C10H16N4O3
Average Molecular Weight: 240.259
Monoisotopic Molecular Weight: 240.122240398
InChI Key: CCLQKVKJOGVQLU-QMMMGPOBSA-N
InChI: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
CAS number: 3650-73-5
IUPAC Name:(2S)-2-(4-aminobutanamido)-3-(1H-imidazol-4-yl)propanoic acid
Traditional IUPAC Name: (2S)-2-(4-aminobutanamido)-3-(1H-imidazol-4-yl)propanoic acid
SMILES:NCCCC(=O)N[C@@H](CC1=CNC=N1)C(O)=O
Chemical Taxonomy
Taxonomy DescriptionThis compound belongs to the class of organic compounds known as hybrid peptides. These are compounds containing at least two different types of amino acids (alpha, beta, gamma, delta) linked to each other through a peptide bond.
Kingdom Organic compounds
Super ClassOrganic acids and derivatives
Class Carboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct Parent Hybrid peptides
Alternative Parents
Substituents
  • Hybrid peptide
  • N-acyl-aliphatic-alpha amino acid
  • N-acyl-alpha amino acid or derivatives
  • N-acyl-alpha-amino acid
  • Gamma amino acid or derivatives
  • N-acyl-l-alpha-amino acid
  • Alpha-amino acid or derivatives
  • N-substituted-alpha-amino acid
  • Imidazolyl carboxylic acid derivative
  • Amino fatty acid
  • Fatty acyl
  • N-acyl-amine
  • Fatty amide
  • Heteroaromatic compound
  • Imidazole
  • Azole
  • Secondary carboxylic acid amide
  • Carboxamide group
  • Azacycle
  • Organoheterocyclic compound
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid amide
  • Hydrocarbon derivative
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Amine
  • Aromatic heteromonocyclic compound
Molecular Framework Aromatic heteromonocyclic compounds
External Descriptors Not Available
Physical Properties
State: Solid
Charge:0
Melting point: Not Available
Experimental Properties:
PropertyValueSource
Predicted Properties
PropertyValueSource
Water Solubility7.37 mg/mLALOGPS
logP-2.9ALOGPS
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 Å2ChemAxon
Rotatable Bond Count7ChemAxon
Refractivity59.75 m3·mol-1ChemAxon
Polarizability24.51 Å3ChemAxon
Number of Rings1ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Biological Properties
Cellular Locations: Cytoplasm
Reactions:
Pathways:
Spectra
Spectra:
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-0udi-1920000000-a03e2c4f8b2c9d6a1635View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-0udi-0920000000-e48ce78a12a5f4834686View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-0fk9-9710000000-32bfa6992d5a80f09a36View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0a4i-0910000000-b804d40537827898f4afView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-03di-5900000000-26897d5ae957a9cb6b35View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-03e9-9300000000-291e9d04c321015b9241View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-000i-0090000000-352076fb2d925fdf8d3dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-0f79-0960000000-6efcaf43e71ab44953faView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-03di-0900000000-4122cf33c566cb50775cView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-03di-4900000000-499817ec7737f4f71264View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-001i-9300000000-5e2839e6bf4597a45ad3View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-0a4l-0980000000-a0e96805123eef741d16View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positivesplash10-0a4i-0900000000-bd852312bcfa93da513dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positivesplash10-03di-2900000000-b0c96a1971d8e230da77View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positivesplash10-03di-6900000000-086dfb0ef83a3e18d4c9View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positivesplash10-03yi-9300000000-cb8906cb50227e7ec5b5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positivesplash10-0a4i-0900000000-e3a22c7580a368d6849bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positivesplash10-03di-0900000000-eab8c998b760a5bc2d46View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positivesplash10-000x-9000000000-d32f932f9d139b180e07View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-0iki-0930000000-80b81755ee1a773ed3bbView in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
References
References:
  • 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
  • Kanehisa, M., Goto, S., Sato, Y., Furumichi, M., Tanabe, M. (2012). "KEGG for integration and interpretation of large-scale molecular data sets." Nucleic Acids Res 40:D109-D114. Pubmed: 22080510
  • 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
  • Lunde HA, Gjessing LR, Sjaastad O: Homocarnosinosis: influence of dietary restriction of histidine. Neurochem Res. 1986 Jun;11(6):825-38. Pubmed: 3736769
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • Promyslov MSh, Mirzoian RA: [Homocarnosine metabolism in brain tissue] Vopr Biokhim Mozga. 1976;11:71-7. Pubmed: 1032224
  • 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
  • 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
  • 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
  • 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
  • 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. Pubmed: 19212411
  • 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
Synthesis Reference: Nagai, Kineshiro; Nagai, Kinuko. Treating inflammatory diseases with homocarnosine. U.S. (1985), 7 pp.
Material Safety Data Sheet (MSDS) Download (PDF)
External Links:
ResourceLink
CHEBI ID491179
HMDB IDHMDB00745
Pubchem Compound ID89235
Kegg IDC00884
ChemSpider ID8418848
Wikipedia IDNot Available
BioCyc IDNot Available

Enzymes

General function:
Involved in transporter activity
Specific function:
Part of the binding-protein-dependent transport system for dipeptides; probably responsible for the translocation of the substrate across the membrane
Gene Name:
dppB
Locus Tag:
PA4503
Molecular weight:
37 kDa
General function:
Involved in transporter activity
Specific function:
Part of the binding-protein-dependent transport system for dipeptides; probably responsible for the translocation of the substrate across the membrane
Gene Name:
dppC
Locus Tag:
PA4504
Molecular weight:
32.4 kDa
General function:
Involved in nucleotide binding
Specific function:
Part of the binding-protein-dependent transport system for dipeptides. Probably responsible for energy coupling to the transport system
Gene Name:
dppD
Locus Tag:
PA4505
Molecular weight:
35.4 kDa
General function:
Involved in nucleotide binding
Specific function:
Part of the binding-protein-dependent transport system for dipeptides. Probably responsible for energy coupling to the transport system
Gene Name:
dppF
Locus Tag:
PA4506
Molecular weight:
36.3 kDa
General function:
Involved in transporter activity
Specific function:
Dipeptide-binding protein of a transport system that can be subject to osmotic shock. DppA is also required for peptide chemotaxis
Gene Name:
dppA
Locus Tag:
PA4496
Molecular weight:
60.1 kDa

Transporters

General function:
Involved in transporter activity
Specific function:
Part of the binding-protein-dependent transport system for dipeptides; probably responsible for the translocation of the substrate across the membrane
Gene Name:
dppB
Locus Tag:
PA4503
Molecular weight:
37 kDa
General function:
Involved in transporter activity
Specific function:
Part of the binding-protein-dependent transport system for dipeptides; probably responsible for the translocation of the substrate across the membrane
Gene Name:
dppC
Locus Tag:
PA4504
Molecular weight:
32.4 kDa
General function:
Involved in nucleotide binding
Specific function:
Part of the binding-protein-dependent transport system for dipeptides. Probably responsible for energy coupling to the transport system
Gene Name:
dppD
Locus Tag:
PA4505
Molecular weight:
35.4 kDa
General function:
Involved in nucleotide binding
Specific function:
Part of the binding-protein-dependent transport system for dipeptides. Probably responsible for energy coupling to the transport system
Gene Name:
dppF
Locus Tag:
PA4506
Molecular weight:
36.3 kDa
General function:
Involved in transporter activity
Specific function:
Dipeptide-binding protein of a transport system that can be subject to osmotic shock. DppA is also required for peptide chemotaxis
Gene Name:
dppA
Locus Tag:
PA4496
Molecular weight:
60.1 kDa