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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2013-05-29 19:24:32 UTC
HMDB IDHMDB00033
Secondary Accession NumbersNone
Metabolite Identification
Common NameCarnosine
DescriptionCarnosine (beta-alanyl-L-histidine) is found exclusively in animal tissues. It is a dipeptide of the amino acids beta-alanine and histidine. Carnosine has the potential to suppress many of the biochemical changes (e.g., protein oxidation, glycation, AGE formation, and cross-linking) that accompany aging and associated pathologies (PMID 16804013 ). It is highly concentrated in muscle and brain tissues. Some autistics patients take it as a dietary supplement, and attribute an improvement in their condition to it. Supplemental carnosine may increase corticosterone levels. This may explain the "hyperactivity" seen in autistic subjects at higher doses. Carnosine also exhibits some antioxidant effects. The antioxidant mechanism of carnosine is attributed to its chelating effect against metal ions, superoxide dismutase (SOD)-like activity, ROS and free radicals scavenging ability (PMID 16406688 ).
Structure
Thumb
Synonyms
  1. b-Alanyl-L-histidine
  2. b-Alanylhistidine
  3. beta-Alanyl-L-histidine
  4. beta-Alanylhistidine
  5. Carnosine
  6. Ignotine
  7. Karnozin
  8. Karnozzn
  9. L-Carnosine
  10. N-(3-Aminopropanoyl)histidine
  11. N-(b-Alanyl)-L-histidine
  12. N-b-Alanyl-L-Histidine
  13. N-beta-Alanyl-L-Histidine
  14. Sevitin
Chemical FormulaC9H14N4O3
Average Molecular Weight226.2325
Monoisotopic Molecular Weight226.106590334
IUPAC Name(2S)-2-(3-aminopropanamido)-3-(1H-imidazol-5-yl)propanoic acid
Traditional Name(2S)-2-(3-aminopropanamido)-3-(3H-imidazol-4-yl)propanoic acid
CAS Registry Number305-84-0
SMILES
NCCC(=O)N[C@@H](CC1=CN=CN1)C(O)=O
InChI Identifier
InChI=1S/C9H14N4O3/c10-2-1-8(14)13-7(9(15)16)3-6-4-11-5-12-6/h4-5,7H,1-3,10H2,(H,11,12)(H,13,14)(H,15,16)/t7-/m0/s1
InChI KeyCQOVPNPJLQNMDC-ZETCQYMHSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassAmino Acids, Peptides, and Analogues
ClassPeptidomimetics
Sub ClassHybrid Peptides
Other Descriptors
  • Aromatic Heteromonocyclic Compounds
Substituents
  • Beta Amino Acid Or Derivative
  • Carboxamide Group
  • Carboxylic Acid
  • 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
Biofunction
  • Component of Histidine metabolism
  • Component of beta-Alanine metabolism
ApplicationNot Available
Cellular locations
  • Cytoplasm
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point253 - 256 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility384 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility11.1 g/LALOGPS
logP-3ALOGPS
logP-4.5ChemAxon
logS-1.3ALOGPS
pKa (Strongest Acidic)3.37ChemAxon
pKa (Strongest Basic)9.13ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count4ChemAxon
Polar Surface Area121.1ChemAxon
Rotatable Bond Count6ChemAxon
Refractivity55.56ChemAxon
Polarizability22.37ChemAxon
Spectra
SpectraMS/MSLC-MS1D NMR2D NMR
Biological Properties
Cellular Locations
  • Cytoplasm
Biofluid Locations
  • Blood
  • Urine
Tissue Location
  • Brain
  • Fibroblasts
  • Intestine
  • Kidney
  • Liver
  • Muscle
  • Neuron
  • Skeletal Muscle
Pathways
NameSMPDB LinkKEGG Link
Beta-Alanine MetabolismSMP00007map00410
Histidine MetabolismSMP00044map00340
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified6.54 +/- 1.0 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified3.1 +/- 7.5 uMInfant (0-1 year old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified1.84 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified1.0 (0.0-2.2) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified5.5 (1.4-12.6) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified19.97 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Shaykhutdinov RA,...
details
UrineDetected and Quantified3.2 +/- 4.36 umol/mmol creatinineChildren (1-13 years old)FemaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified3.5 +/- 2.7 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified4.0 +/- 2.5 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified1.2 (0.2-4.1) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified1.1+/-0.5 umol/mmol creatinineNot SpecifiedBothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified3.28 +/- 0.91 uMElderly (>65 years old)BothAlzheimer's disease details
UrineDetected and Quantified84.8 (56.5-113.0) umol/mmol creatinineChildren (1-13 years old)BothCarnosinuria details
UrineDetected and Quantified18.8 +/- 10.31 umol/mmol creatinineAdult (>18 years old)BothAlzheimer's disease details
UrineDetected and Quantified56.56-113.1 umol/mmol creatinineAdult (>18 years old)Not SpecifiedCarnosinuria details
Associated Disorders and Diseases
Disease References
Alzheimer's disease
  1. Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. Pubmed: 17031479
Carnosinuria
  1. Furst P: Amino acid metabolism in uremia. J Am Coll Nutr. 1989 Aug;8(4):310-23. Pubmed: 2674258
  2. MetaGene
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB000511
KNApSAcK IDNot Available
Chemspider ID388363
KEGG Compound IDC00386
BioCyc IDCARNOSINE
BiGG ID1800369
Wikipedia LinkCarnosine
NuGOwiki LinkHMDB00033
Metagene LinkHMDB00033
METLIN ID38
PubChem Compound439224
PDB IDNot Available
ChEBI ID15727
References
Synthesis Reference Vezenkov, L.; Yanachkov, O. Synthesis of carnosine by trimethylsilyl protection. Dokladi na Bulgarskata Akademiya na Naukite (1991), 44(8), 53-6
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Park YJ, Volpe SL, Decker EA: Quantitation of carnosine in humans plasma after dietary consumption of beef. J Agric Food Chem. 2005 Jun 15;53(12):4736-9. Pubmed: 15941308
  2. Guiotto A, Calderan A, Ruzza P, Borin G: Carnosine and carnosine-related antioxidants: a review. Curr Med Chem. 2005;12(20):2293-315. Pubmed: 16181134
  3. Hipkiss AR, Preston JE, Himsworth DT, Worthington VC, Keown M, Michaelis J, Lawrence J, Mateen A, Allende L, Eagles PA, Abbott NJ: Pluripotent protective effects of carnosine, a naturally occurring dipeptide. Ann N Y Acad Sci. 1998 Nov 20;854:37-53. Pubmed: 9928418
  4. Guiotto A, Calderan A, Ruzza P, Osler A, Rubini C, Jo DG, Mattson MP, Borin G: Synthesis and evaluation of neuroprotective alpha,beta-unsaturated aldehyde scavenger histidyl-containing analogues of carnosine. J Med Chem. 2005 Sep 22;48(19):6156-61. Pubmed: 16162015
  5. Hipkiss AR, Brownson C: Carnosine reacts with protein carbonyl groups: another possible role for the anti-ageing peptide? Biogerontology. 2000;1(3):217-23. Pubmed: 11707898
  6. Furst P: Amino acid metabolism in uremia. J Am Coll Nutr. 1989 Aug;8(4):310-23. Pubmed: 2674258
  7. Lee YT, Hsu CC, Lin MH, Liu KS, Yin MC: Histidine and carnosine delay diabetic deterioration in mice and protect human low density lipoprotein against oxidation and glycation. Eur J Pharmacol. 2005 Apr 18;513(1-2):145-50. Epub 2005 Apr 2. Pubmed: 15878720
  8. Hipkiss AR, Brownson C, Bertani MF, Ruiz E, Ferro A: Reaction of carnosine with aged proteins: another protective process? Ann N Y Acad Sci. 2002 Apr;959:285-94. Pubmed: 11976203
  9. Mannion AF, Jakeman PM, Dunnett M, Harris RC, Willan PL: Carnosine and anserine concentrations in the quadriceps femoris muscle of healthy humans. Eur J Appl Physiol Occup Physiol. 1992;64(1):47-50. Pubmed: 1735411
  10. Hipkiss AR, Brownson C, Carrier MJ: Carnosine, the anti-ageing, anti-oxidant dipeptide, may react with protein carbonyl groups. Mech Ageing Dev. 2001 Sep 15;122(13):1431-45. Pubmed: 11470131
  11. Suzuki Y, Ito O, Mukai N, Takahashi H, Takamatsu K: High level of skeletal muscle carnosine contributes to the latter half of exercise performance during 30-s maximal cycle ergometer sprinting. Jpn J Physiol. 2002 Apr;52(2):199-205. Pubmed: 12139778
  12. Kang JH, Kim KS, Choi SY, Kwon HY, Won MH, Kang TC: Protection by carnosine-related dipeptides against hydrogen peroxide-mediated ceruloplasmin modification. Mol Cells. 2002 Feb 28;13(1):107-12. Pubmed: 11911459
  13. Willi SM, Zhang Y, Hill JB, Phelan MC, Michaelis RC, Holden KR: A deletion in the long arm of chromosome 18 in a child with serum carnosinase deficiency. Pediatr Res. 1997 Feb;41(2):210-3. Pubmed: 9029640
  14. Tallon MJ, Harris RC, Boobis LH, Fallowfield JL, Wise JA: The carnosine content of vastus lateralis is elevated in resistance-trained bodybuilders. J Strength Cond Res. 2005 Nov;19(4):725-9. Pubmed: 16287364
  15. Mozdzan M, Szemraj J, Rysz J, Nowak D: Antioxidant properties of carnosine re-evaluated with oxidizing systems involving iron and copper ions. Basic Clin Pharmacol Toxicol. 2005 May;96(5):352-60. Pubmed: 15853927
  16. Jackson MC, Lenney JF: The distribution of carnosine and related dipeptides in rat and human tissues. Inflamm Res. 1996 Mar;45(3):132-5. Pubmed: 8689392
  17. Hipkiss AR: Would carnosine or a carnivorous diet help suppress aging and associated pathologies? Ann N Y Acad Sci. 2006 May;1067:369-74. Pubmed: 16804013
  18. Guney Y, Turkcu UO, Hicsonmez A, Andrieu MN, Guney HZ, Bilgihan A, Kurtman C: Carnosine may reduce lung injury caused by radiation therapy. Med Hypotheses. 2006;66(5):957-9. Epub 2006 Jan 10. Pubmed: 16406688

Enzymes

General function:
Involved in peroxidase activity
Specific function:
Part of the host defense system of polymorphonuclear leukocytes. It is responsible for microbicidal activity against a wide range of organisms. In the stimulated PMN, MPO catalyzes the production of hypohalous acids, primarily hypochlorous acid in physiologic situations, and other toxic intermediates that greatly enhance PMN microbicidal activity.
Gene Name:
MPO
Uniprot ID:
P05164
Molecular weight:
83867.71
References
  1. Boldyrev A, Abe H, Stvolinsky S, Tyulina O: Effects of carnosine and related compounds on generation of free oxygen species: a comparative study. Comp Biochem Physiol B Biochem Mol Biol. 1995 Nov;112(3):481-5. Pubmed: 8529024
General function:
Involved in metallopeptidase activity
Specific function:
Preferential hydrolysis of the beta-Ala-|-His dipeptide (carnosine), and also anserine, Xaa-|-His dipeptides and other dipeptides including homocarnosine
Gene Name:
CNDP1
Uniprot ID:
Q96KN2
Molecular weight:
56691.6
General function:
Involved in carboxy-lyase activity
Specific function:
Catalyzes the biosynthesis of histamine from histidine.
Gene Name:
HDC
Uniprot ID:
P19113
Molecular weight:
74139.825
References
  1. Nagai K, Suda T, Kawasaki K, Mathuura S: Action of carnosine and beta-alanine on wound healing. Surgery. 1986 Nov;100(5):815-21. Pubmed: 3095942
General function:
Involved in structural molecule activity
Specific function:
Vimentins are class-III intermediate filaments found in various non-epithelial cells, especially mesenchymal cells
Gene Name:
VIM
Uniprot ID:
P08670
Molecular weight:
53651.2
References
  1. Hipkiss AR: Could carnosine or related structures suppress Alzheimer's disease? J Alzheimers Dis. 2007 May;11(2):229-40. Pubmed: 17522447
General function:
Involved in ATP binding
Specific function:
In cooperation with other chaperones, Hsp70s stabilize preexistent proteins against aggregation and mediate the folding of newly translated polypeptides in the cytosol as well as within organelles. These chaperones participate in all these processes through their ability to recognize nonnative conformations of other proteins. They bind extended peptide segments with a net hydrophobic character exposed by polypeptides during translation and membrane translocation, or following stress-induced damage. In case of rotavirus A infection, serves as a post-attachment receptor for the virus to facilitate entry into the cell
Gene Name:
HSPA1A
Uniprot ID:
P08107
Molecular weight:
70051.6
References
  1. Wada I, Otaka M, Jin M, Odashima M, Komatsu K, Konishi N, Matsuhashi T, Horikawa Y, Ohba R, Itoh H, Watanabe S: Expression of HSP72 in the gastric mucosa is regulated by gastric acid in rats-correlation of HSP72 expression with mucosal protection. Biochem Biophys Res Commun. 2006 Oct 20;349(2):611-8. Epub 2006 Aug 23. Pubmed: 16945336
General function:
Involved in catalytic activity
Specific function:
Catalyzes the synthesis of carnosine and homocarnosine. Carnosine is synthesized more efficiently than homocarnosine.
Gene Name:
CARNS1
Uniprot ID:
A5YM72
Molecular weight:
101318.725
Reactions
Adenosine triphosphate + L-Histidine + Beta-Alanine → ADP + Phosphoric acid + Carnosinedetails
Adenosine triphosphate + L-Histidine + Beta-Alanine → Adenosine monophosphate + Pyrophosphate + Carnosinedetails

Transporters

General function:
Involved in transporter activity
Specific function:
Proton-coupled intake of oligopeptides of 2 to 4 amino acids with a preference for dipeptides
Gene Name:
SLC15A2
Uniprot ID:
Q16348
Molecular weight:
81782.8
References
  1. Teuscher NS, Keep RF, Smith DE: PEPT2-mediated uptake of neuropeptides in rat choroid plexus. Pharm Res. 2001 Jun;18(6):807-13. Pubmed: 11474785