Human Metabolome Database Version 3.5

Showing metabocard for Homocarnosine (HMDB00745)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-05-29 13:28:53 -0600
Secondary Accession Numbers None
Metabolite Identification
Common Name Homocarnosine
Description Homocarnosine is a normal human metabolite, the brain-specific dipeptide of gamma-aminobutyric acid (GABA) and histidine. (PMID 1266573 Link_out). Increased concentration of CSF homocarnosine has been found in familial spastic paraplegia. (PMID 842287 Link_out). Homocarnosinosis (an inherited disorder, OMIM 236130 Link_out) 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 Link_out). In glial tumors of human brain the content of homocarnosine has been found to be lower than in brain tissue (PMID 1032224 Link_out), while an increase in content of homocarnosine was observed in brain tissue of animals under experimental trauma of cranium. (PMID 1025883 Link_out).
Structure Thumb
Download: MOL | SDF | PDB | SMILES | InChI
Display: 2D Structure | 3D Structure
  1. g-Aminobutyryl-L-histidine
  2. g-Aminobutyrylhistidine
  3. gamma-Aminobutyryl-L-histidine
  4. gamma-Aminobutyrylhistidine
  5. Homocarnosine
  6. L-Homocarnosine
  7. N-(4-Aminobutyryl)-Histidine
  8. N-(4-Aminobutyryl)-L-Histidine
Chemical Formula C10H16N4O3
Average Molecular Weight 240.259
Monoisotopic Molecular Weight 240.122240398
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
CAS Registry Number 3650-73-5
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
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Amino Acids, Peptides, and Analogues
Class Peptidomimetics
Sub Class Hybrid Peptides
Other Descriptors
  • Aromatic Heteromonocyclic Compounds
  • Alpha Amino Acid Or Derivative
  • Carboxamide Group
  • Carboxylic Acid
  • Gamma Amino Acid Or Derivative
  • Imidazole
  • Imidazolyl Carboxylic Acid Derivative
  • N Acyl Alpha Amino Acid
  • N Substituted Alpha Amino Acid
  • Primary Aliphatic Amine (Alkylamine)
  • Secondary Carboxylic Acid Amide
Direct Parent Hybrid Peptides
Status Detected and Quantified
  • Endogenous
Biofunction Not Available
Application Not Available
Cellular locations
  • Cytoplasm (predicted from logP)
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point Not Available Not Available
Boiling Point Not Available Not Available
Water Solubility Not Available Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
Water Solubility 7.37 g/L ALOGPS
LogP -2.85 ALOGPS
LogP -3.9 ChemAxon
LogS -1.51 ALOGPS
pKa (strongest acidic) 3.45 ChemAxon
pKa (strongest basic) 9.99 ChemAxon
Hydrogen Acceptor Count 5 ChemAxon
Hydrogen Donor Count 4 ChemAxon
Polar Surface Area 121.1 A2 ChemAxon
Rotatable Bond Count 7 ChemAxon
Refractivity 59.75 ChemAxon
Polarizability 24.51 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge 0 ChemAxon
1H NMR Spectrum
MS/MS Spectrum Quattro_QQQ 10
MS/MS Spectrum Quattro_QQQ 25
MS/MS Spectrum Quattro_QQQ 40
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 10
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 20
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 30
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 40
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 50
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 10
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 20
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 30
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 40
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 50
MS/MS Spectrum LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies)
MS/MS Spectrum LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies)
[1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Location
  • Brain
  • Prostate
Pathways Not Available
Normal Concentrations
Biofluid Status Value Age Sex Condition Reference
Cerebrospinal Fluid (CSF) Detected and Quantified
2.70 +/- 2.50 uM Adult (>18 years old) Both Normal
  • Geigy Scient...
Urine Detected and Quantified
0.26 umol/mmol creatinine Adult (>18 years old) Both Normal
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease References None
Associated OMIM IDs None
DrugBank ID Not Available
DrugBank Metabolite ID Not Available
Phenol Explorer Compound ID Not Available
Phenol Explorer Metabolite ID Not Available
FoodDB ID FDB022217
KNApSAcK ID Not Available
Chemspider ID 8418848 Link_out
KEGG Compound ID C00884 Link_out
BioCyc ID Not Available
BiGG ID Not Available
Wikipedia Link Not Available
NuGOwiki Link HMDB00745 Link_out
Metagene Link HMDB00745 Link_out
METLIN ID 270 Link_out
PubChem Compound 10243361 Link_out
PDB ID Not Available
ChEBI ID 491179 Link_out
Synthesis Reference Nagai, Kineshiro; Nagai, Kinuko. Treating inflammatory diseases with homocarnosine. U.S. (1985), 7 pp.
Material Safety Data Sheet (MSDS) Download (PDF)
General References
  1. 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 Link_out
  2. 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 Link_out
  3. 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 Link_out
  4. 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 Link_out
  5. 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 Link_out
  6. 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 Link_out
  7. Promyslov MSh, Mirzoian RA: [Homocarnosine metabolism in brain tissue] Vopr Biokhim Mozga. 1976;11:71-7. Pubmed: 1032224 Link_out
  8. 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 Link_out
  9. 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 Link_out
  10. 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 Link_out
  11. 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 Link_out
  12. 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 Link_out
  13. 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 Link_out
  14. 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 Link_out
  15. 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 Link_out
  16. Lunde HA, Gjessing LR, Sjaastad O: Homocarnosinosis: influence of dietary restriction of histidine. Neurochem Res. 1986 Jun;11(6):825-38. Pubmed: 3736769 Link_out

Name: Beta-Ala-His dipeptidase
Reactions: Not Available
Gene Name: CNDP1
Uniprot ID: Q96KN2 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Carnosine synthase 1
Adenosine triphosphate + L-Histidine + Gamma-Aminobutyric acid unknown Adenosine monophosphate + Pyrophosphate + Homocarnosine details
Gene Name: CARNS1
Uniprot ID: A5YM72 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA