Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2013-05-29 19:28:53 UTC
HMDB IDHMDB00745
Secondary Accession NumbersNone
Metabolite 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
  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 FormulaC10H16N4O3
Average Molecular Weight240.259
Monoisotopic Molecular Weight240.122240398
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 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
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassAmino Acids, Peptides, and Analogues
ClassPeptidomimetics
Sub ClassHybrid Peptides
Other Descriptors
  • Aromatic Heteromonocyclic Compounds
Substituents
  • 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 ParentHybrid Peptides
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locations
  • Cytoplasm (predicted from logP)
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-2.9ALOGPS
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 area121.1ChemAxon
rotatable bond count7ChemAxon
refractivity59.75ChemAxon
polarizability24.51ChemAxon
Spectra
Spectra
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Location
  • Brain
  • Prostate
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReference
Cerebrospinal Fluid (CSF)Detected and Quantified2.70 +/- 2.50 uMAdult (>18 years old)BothNormal
  • Geigy Scient...
UrineDetected and Quantified0.26 umol/mmol creatinineAdult (>18 years old)BothNormal
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022217
KNApSAcK IDNot Available
Chemspider ID8418848
KEGG Compound IDC00884
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00745
Metagene LinkHMDB00745
METLIN ID270
PubChem Compound10243361
PDB IDNot Available
ChEBI ID491179
References
Synthesis ReferenceNagai, Kineshiro; Nagai, Kinuko. Treating inflammatory diseases with homocarnosine. U.S. (1985), 7 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
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. 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

Enzymes

Gene Name:
CNDP1
Uniprot ID:
Q96KN2
Gene Name:
CARNS1
Uniprot ID:
A5YM72
Reactions
Adenosine triphosphate + L-Histidine + Gamma-Aminobutyric acid unknown Adenosine monophosphate + Pyrophosphate + Homocarnosinedetails