Record Information
Version2.0
Creation Date2012-05-31 13:00:58 -0600
Update Date2015-09-13 12:56:09 -0600
Secondary Accession Numbers
  • ECMDB00745
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
Weight:Average: 240.259
Monoisotopic: 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
DescriptionThis compound belongs to the class of chemical entities 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.
KingdomChemical entities
Super ClassOrganic compounds
ClassOrganic acids and derivatives
Sub ClassPeptidomimetics
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
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:
SMPDB Pathways:
inner membrane transportPW000786 Pw000786Pw000786 greyscalePw000786 simple
KEGG Pathways:
  • Arginine and proline metabolism ec00330
EcoCyc Pathways:Not Available
Concentrations
Not Available
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
GC-MSGC-MS Spectrum - GC-MSsplash10-001i-9410000000-343b9389cd3a9e7656c5View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOFsplash10-0udi-1920000000-a03e2c4f8b2c9d6a1635View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOFsplash10-0udi-0920000000-e48ce78a12a5f4834686View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOFsplash10-0fk9-9710000000-32bfa6992d5a80f09a36View in MoNA
GC-MSGC-MS Spectrum - GC-MS (1 TMS)splash10-000t-9620000000-f0df8a34f8155fd39194View 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
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-000i-0090000000-5b43effb3dfed505b61bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0f79-0960000000-52b5a3c482216dc5067dView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-00dl-1490000000-abafa225b4c4cc6f1314View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0bti-5930000000-d1e289dfa041a96af6e4View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-052f-9400000000-3dc5fad146dc39014458View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-000i-0490000000-dfebe908fd4392611bb6View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0uej-3940000000-c7f4d398999fc797b544View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0f7o-9500000000-f8ed9a520a7d500d0c1eView 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 ID85981
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
Uniprot ID:
P0AEF8
Molecular weight:
37497
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
Uniprot ID:
P0AEG1
Molecular weight:
32308
General function:
Involved in transporter activity
Specific function:
Part of the binding-protein-dependent transport system for oligopeptides; probably responsible for the translocation of the substrate across the membrane
Gene Name:
oppB
Uniprot ID:
P0AFH2
Molecular weight:
33443
General function:
Involved in transporter activity
Specific function:
Part of the binding-protein-dependent transport system for oligopeptides; probably responsible for the translocation of the substrate across the membrane
Gene Name:
oppC
Uniprot ID:
P0AFH6
Molecular weight:
33022
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
Uniprot ID:
P0AAG0
Molecular weight:
35844
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
Uniprot ID:
P37313
Molecular weight:
37560
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
Uniprot ID:
P23847
Molecular weight:
60293

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
Uniprot ID:
P0AEF8
Molecular weight:
37497
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
Uniprot ID:
P0AEG1
Molecular weight:
32308
General function:
Involved in transporter activity
Specific function:
Part of the binding-protein-dependent transport system for oligopeptides; probably responsible for the translocation of the substrate across the membrane
Gene Name:
oppB
Uniprot ID:
P0AFH2
Molecular weight:
33443
General function:
Involved in transporter activity
Specific function:
Part of the binding-protein-dependent transport system for oligopeptides; probably responsible for the translocation of the substrate across the membrane
Gene Name:
oppC
Uniprot ID:
P0AFH6
Molecular weight:
33022
General function:
Involved in transporter activity
Specific function:
Involved in a peptide intake transport system that plays a role in the resistance to antimicrobial peptides
Gene Name:
sapB
Uniprot ID:
P0AGH3
Molecular weight:
36038
General function:
Involved in transporter activity
Specific function:
Involved in a peptide intake transport system that plays a role in the resistance to antimicrobial peptides
Gene Name:
sapC
Uniprot ID:
P0AGH5
Molecular weight:
31548
General function:
Involved in peptide transporter activity
Specific function:
Proton-dependent permease that transports di- and tripeptides. Has a clear preference for dipeptides and tripeptides composed of L-amino acids, and discriminates dipeptides on the basis of the position of charges within the substrate
Gene Name:
dtpB
Uniprot ID:
P36837
Molecular weight:
53575
General function:
Involved in peptide transporter activity
Specific function:
Probable proton-dependent permease that transports di- and tripeptides. Shows significantly higher specificity towards dipeptides
Gene Name:
yjdL
Uniprot ID:
P39276
Molecular weight:
53054
General function:
Involved in peptide transporter activity
Specific function:
Probable proton-dependent permease that transports dipeptides
Gene Name:
dtpD
Uniprot ID:
P75742
Molecular weight:
54158
General function:
Involved in peptide transporter activity
Specific function:
Proton-dependent permease that transports di- and tripeptides as well as structurally related peptidomimetics such as aminocephalosporins into the cell. Has a clear preference for dipeptides and tripeptides composed of L-amino acids, and discriminates dipeptides on the basis of the position of charges within the substrate
Gene Name:
dtpA
Uniprot ID:
P77304
Molecular weight:
53991
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
Uniprot ID:
P0AAG0
Molecular weight:
35844
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
Uniprot ID:
P37313
Molecular weight:
37560
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
Uniprot ID:
P23847
Molecular weight:
60293