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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-10-18 23:06:57 UTC
HMDB IDHMDB00064
Secondary Accession NumbersNone
Metabolite Identification
Common NameCreatine
DescriptionCreatine is an amino acid that occurs in vertebrate tissues and in urine. In muscle tissue, creatine generally occurs as phosphocreatine. Creatine is excreted as creatinine in the urine. Creatine functions as part of the cell's energy shuttle. The high energy phosphate group of ATP is transferred to creatine to form phosphocreatine in the following reaction: Cr + ATP <-> PCr + ADP. This reaction is reversibly catalyzed by creatine kinase. In the human body creatine is synthesized mainly in the liver by the use of parts from three different amino acids - arginine, glycine, and methionine. 95% of it is later stored in the skeletal muscles, with the rest in the brain, heart, testes.
Structure
Thumb
Synonyms
ValueSource
((amino(imino)Methyl)(methyl)amino)acetic acidChEBI
(alpha-methylguanido)Acetic acidChEBI
(N-methylcarbamimidamido)Acetic acidChEBI
alpha-methylguanidino Acetic acidChEBI
CreatinChEBI
KreatinChEBI
MethylglycocyamineChEBI
N-(Aminoiminomethyl)-N-methylglycineChEBI
N-[(e)-amino(imino)METHYL]-N-methylglycineChEBI
N-AmidinosarcosineChEBI
N-Carbamimidoyl-N-methylglycineChEBI
N-Methyl-N-guanylglycineChEBI
((amino(imino)Methyl)(methyl)amino)acetateGenerator
(a-methylguanido)AcetateGenerator
(a-methylguanido)Acetic acidGenerator
(alpha-methylguanido)AcetateGenerator
(α-methylguanido)acetateGenerator
(α-methylguanido)acetic acidGenerator
(N-methylcarbamimidamido)AcetateGenerator
a-methylguanidino AcetateGenerator
a-methylguanidino Acetic acidGenerator
alpha-methylguanidino AcetateGenerator
α-methylguanidino acetateGenerator
α-methylguanidino acetic acidGenerator
Cosmocair C 100HMDB
Creatine hydrateHMDB
KrebiozonHMDB
MethylguanidoacetateHMDB
Methylguanidoacetic acidHMDB
N-(Aminoiminomethyl)-N-methyl-glycineHMDB
PhosphagenHMDB
[[amino(imino)Methyl](methyl)amino]acetateHMDB
[[amino(imino)Methyl](methyl)amino]acetic acidHMDB
Chemical FormulaC4H9N3O2
Average Molecular Weight131.1332
Monoisotopic Molecular Weight131.069476547
IUPAC Name2-(1-methylcarbamimidamido)acetic acid
Traditional Namecreatine
CAS Registry Number57-00-1
SMILES
CN(CC(O)=O)C(N)=N
InChI Identifier
InChI=1S/C4H9N3O2/c1-7(4(5)6)2-3(8)9/h2H2,1H3,(H3,5,6)(H,8,9)
InChI KeyInChIKey=CVSVTCORWBXHQV-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as alpha amino acids and derivatives. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon), or a derivative thereof.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentAlpha amino acids and derivatives
Alternative Parents
Substituents
  • Alpha-amino acid or derivatives
  • Tertiary amine
  • Guanidine
  • Carboximidamide
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Imine
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
Biofunction
  • Component of Arginine and proline metabolism
  • Component of Glycine, serine and threonine metabolism
ApplicationNot Available
Cellular locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point303 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility13.3 mg/mL at 18 °CNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility4.11 mg/mLALOGPS
logP-1.6ALOGPS
logP-2.9ChemAxon
logS-1.5ALOGPS
pKa (Strongest Acidic)3.5ChemAxon
pKa (Strongest Basic)12.43ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area90.41 Å2ChemAxon
Rotatable Bond Count2ChemAxon
Refractivity42.01 m3·mol-1ChemAxon
Polarizability12.17 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-000x-9800000000-bc1387ab36f71e04b988View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0006-9000000000-6c686da36a88d9fbdbcfView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0006-9000000000-13b7d28e8f5be1c5dadaView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-cc4bd4c587dd80f63bcbView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-0udi-3900000000-d35c7578ad50de8377b7View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-a4d7fee14f74e1ed8b4bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-e5d62520dfcbf4bfe5c9View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-06fbcf897ccce3fa90b7View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-9000000000-9a0b8275e74605a92681View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000f-9000000000-5f622f2c3de4f213a8edView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-20ba65c7a2c9434fcf39View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-01qc-0965022201-53bb6f168e0934ae4a87View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-000i-9000000000-638479794e8c3a6e0b61View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-004i-0900000000-39676937b1a3ad0dc0bcView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-03di-0090000000-d53c75786742ecdb3c16View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-001l-0945022201-2fc3f234454021c5e202View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-000i-9000000000-40aa07f89dddb181e489View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-001i-0900000000-5aca4d0b8ed7d2500af2View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-03di-0190000000-9a9685c32bf6c721a497View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-001i-1900000000-c99f9492e0b84d5fae6bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positivesplash10-0006-9300000000-8ab609e6dd62bd18841fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positivesplash10-0006-9000000000-e04e407d5f06d21582c4View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positivesplash10-0006-9000000000-fd81d06317727fa740b1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positivesplash10-0006-9000000000-8428e2af90720afb4337View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positivesplash10-001i-0900000000-3162b4c94e66796a643bView in MoNA
MSMass Spectrum (Electron Ionization)splash10-0006-9100000000-47f885347545055f546dView in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,1H] 2D NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
Biofluid Locations
  • Blood
  • Breast Milk
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Location
  • Adipose Tissue
  • Bladder
  • Brain
  • Epidermis
  • Fibroblasts
  • Heart
  • Intestine
  • Kidney
  • Muscle
  • Myelin
  • Nerve Cells
  • Neuron
  • Placenta
  • Platelet
  • Prostate
  • Skeletal Muscle
  • Spleen
  • Testes
Pathways
NameSMPDB LinkKEGG Link
3-Phosphoglycerate dehydrogenase deficiencySMP00721Not Available
Arginine and Proline MetabolismSMP00020map00330
Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency)SMP00362Not Available
Creatine deficiency, guanidinoacetate methyltransferase deficiencySMP00504Not Available
Dihydropyrimidine Dehydrogenase Deficiency (DHPD)SMP00179Not Available
Dimethylglycine Dehydrogenase DeficiencySMP00242Not Available
Dimethylglycine Dehydrogenase DeficiencySMP00484Not Available
Glycine and Serine MetabolismSMP00004map00260
Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency)SMP00188Not Available
Hyperglycinemia, non-ketoticSMP00485Not Available
Hyperornithinemia with gyrate atrophy (HOGA)SMP00505Not Available
Hyperornithinemia-hyperammonemia-homocitrullinuria [HHH-syndrome]SMP00506Not Available
Hyperprolinemia Type ISMP00361Not Available
Hyperprolinemia Type IISMP00360Not Available
L-arginine:glycine amidinotransferase deficiencySMP00507Not Available
Non Ketotic HyperglycinemiaSMP00223Not Available
Ornithine Aminotransferase Deficiency (OAT Deficiency)SMP00363Not Available
Prolidase Deficiency (PD)SMP00207Not Available
Prolinemia Type IISMP00208Not Available
SarcosinemiaSMP00244Not Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified54.8 +/- 21.0 uMAdult (>18 years old)FemaleNormal details
BloodDetected and Quantified30.1 +/- 12.3 uMAdult (>18 years old)MaleNormal details
BloodDetected and Quantified84.0 +/- 23.2 uMNewborn (0-30 days old)Not SpecifiedNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified53.0 (17.0 - 82.0) uMChildren (1-13 years old)Not SpecifiedNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified32.22 +/- 16.42 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified36.7 +/- 28.3 uMAdult (>18 years old)BothNormal details
Breast MilkDetected and Quantified51.6 +/- 24.5 uMAdult (>18 years old)Female
Normal
details
Cerebrospinal Fluid (CSF)Detected and Quantified44 +/- 13 uMAdult (>18 years old)BothNormal details
SalivaDetected and Quantified8.32 +/- 3.30 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified30.12 +/- 18.36 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected and Quantified23.2 +/- 29.4 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified30.7 +/- 14.6 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified33.9 +/- 45.9 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified25.94 +/- 9.75 uMAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified113.0 (0.00-654.0) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified113(0-654) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.00-404.37 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
details
UrineDetected and Quantified10-1350 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified46 (3-448) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified26.0 (5.0-95.0) umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified46.0 (9.0-135.0) umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified11.30 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Shaykhutdinov RA,...
details
UrineDetected and Quantified497.145 +/- 435.142 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified33.8 +/- 37.7 uMAdult (>18 years old)BothHeart Transplant details
BloodDetected and Quantified40.3 (28.3-56.6) uMAdult (>18 years old)FemaleCirrhosis details
BloodDetected and Quantified25.7 (16.5 - 30.8) uMAdult (>18 years old)MaleCirrhosis details
BloodDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothColorectal cancer details
BloodDetected and Quantified43.2 +/- 18.10 uMAdult (>18 years old)Both
Schizophrenia
details
BloodDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Pancreatic
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Irritable Bowel Syndrome
details
SalivaDetected and Quantified29.86 +/- 19.99 uMAdult (>18 years old)MaleAlzheimer's disease details
SalivaDetected and Quantified26.61 +/- 5.64 uMAdult (>18 years old)MaleFrontotemporal lobe dementia details
SalivaDetected and Quantified19.48 +/- 12.88 uMAdult (>18 years old)BothLewy body disease details
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Schizophrenia
details
UrineDetected and Quantified37.0 (0.0-197.0) umol/mmol creatinineNot SpecifiedBothLung cancer details
UrineDetected and Quantified1540 umol/mmol creatinineAdult (>18 years old)FemaleRhabdomyolysis details
UrineDetected and Quantified233.588 +/- 539.846 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
Associated Disorders and Diseases
Disease References
Cirrhosis
  1. Marescau B, De Deyn PP, Holvoet J, Possemiers I, Nagels G, Saxena V, Mahler C: Guanidino compounds in serum and urine of cirrhotic patients. Metabolism. 1995 May;44(5):584-8. [7752905 ]
Schizophrenia
  1. Koike S, Bundo M, Iwamoto K, Suga M, Kuwabara H, Ohashi Y, Shinoda K, Takano Y, Iwashiro N, Satomura Y, Nagai T, Natsubori T, Tada M, Yamasue H, Kasai K: A snapshot of plasma metabolites in first-episode schizophrenia: a capillary electrophoresis time-of-flight mass spectrometry study. Transl Psychiatry. 2014 Apr 8;4:e379. doi: 10.1038/tp.2014.19. [24713860 ]
  2. Cai HL, Li HD, Yan XZ, Sun B, Zhang Q, Yan M, Zhang WY, Jiang P, Zhu RH, Liu YP, Fang PF, Xu P, Yuan HY, Zhang XH, Hu L, Yang W, Ye HS: Metabolomic analysis of biochemical changes in the plasma and urine of first-episode neuroleptic-naive schizophrenia patients after treatment with risperidone. J Proteome Res. 2012 Aug 3;11(8):4338-50. doi: 10.1021/pr300459d. Epub 2012 Jul 26. [22800120 ]
Lung Cancer
  1. Stretch C, Eastman T, Mandal R, Eisner R, Wishart DS, Mourtzakis M, Prado CM, Damaraju S, Ball RO, Greiner R, Baracos VE: Prediction of skeletal muscle and fat mass in patients with advanced cancer using a metabolomic approach. J Nutr. 2012 Jan;142(1):14-21. doi: 10.3945/jn.111.147751. Epub 2011 Dec 7. [22157537 ]
Rhabdomyolysis
  1. Bairaktari E, Seferiadis K, Liamis G, Psihogios N, Tsolas O, Elisaf M: Rhabdomyolysis-related renal tubular damage studied by proton nuclear magnetic resonance spectroscopy of urine. Clin Chem. 2002 Jul;48(7):1106-9. [12089184 ]
Associated OMIM IDs
DrugBank IDDB00148
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB005403
KNApSAcK IDNot Available
Chemspider ID566
KEGG Compound IDC00300
BioCyc IDCREATINE
BiGG ID34543
Wikipedia LinkCreatine
NuGOwiki LinkHMDB00064
Metagene LinkHMDB00064
METLIN ID7
PubChem Compound586
PDB IDCRN
ChEBI ID16919
References
Synthesis ReferenceThalhammer, Franz; Gastner, Thomas. Preparation of creatine, creatine monohydrate and guanidinoacetic acid. Ger. Offen. (2007), 5pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Subramanian A, Gupta A, Saxena S, Gupta A, Kumar R, Nigam A, Kumar R, Mandal SK, Roy R: Proton MR CSF analysis and a new software as predictors for the differentiation of meningitis in children. NMR Biomed. 2005 Jun;18(4):213-25. [15627241 ]
  2. Bales JR, Higham DP, Howe I, Nicholson JK, Sadler PJ: Use of high-resolution proton nuclear magnetic resonance spectroscopy for rapid multi-component analysis of urine. Clin Chem. 1984 Mar;30(3):426-32. [6321058 ]
  3. Schonberger B: [Overactive bladder--which diagnosis investigations are necessary before initiating primary treatment?] Urologe A. 2003 Jun;42(6):787-92. Epub 2003 Apr 25. [12851769 ]
  4. Mercimek-Mahmutoglu S, Stoeckler-Ipsiroglu S, Adami A, Appleton R, Araujo HC, Duran M, Ensenauer R, Fernandez-Alvarez E, Garcia P, Grolik C, Item CB, Leuzzi V, Marquardt I, Muhl A, Saelke-Kellermann RA, Salomons GS, Schulze A, Surtees R, van der Knaap MS, Vasconcelos R, Verhoeven NM, Vilarinho L, Wilichowski E, Jakobs C: GAMT deficiency: features, treatment, and outcome in an inborn error of creatine synthesis. Neurology. 2006 Aug 8;67(3):480-4. Epub 2006 Jul 19. [16855203 ]
  5. McConell GK, Shinewell J, Stephens TJ, Stathis CG, Canny BJ, Snow RJ: Creatine supplementation reduces muscle inosine monophosphate during endurance exercise in humans. Med Sci Sports Exerc. 2005 Dec;37(12):2054-61. [16331129 ]
  6. McMorris T, Harris RC, Swain J, Corbett J, Collard K, Dyson RJ, Dye L, Hodgson C, Draper N: Effect of creatine supplementation and sleep deprivation, with mild exercise, on cognitive and psychomotor performance, mood state, and plasma concentrations of catecholamines and cortisol. Psychopharmacology (Berl). 2006 Mar;185(1):93-103. Epub 2006 Jan 17. [16416332 ]
  7. Wang PF, McLeish MJ, Kneen MM, Lee G, Kenyon GL: An unusually low pK(a) for Cys282 in the active site of human muscle creatine kinase. Biochemistry. 2001 Oct 2;40(39):11698-705. [11570870 ]
  8. Olsen S, Aagaard P, Kadi F, Tufekovic G, Verney J, Olesen JL, Suetta C, Kjaer M: Creatine supplementation augments the increase in satellite cell and myonuclei number in human skeletal muscle induced by strength training. J Physiol. 2006 Jun 1;573(Pt 2):525-34. Epub 2006 Mar 31. [16581862 ]
  9. Ellington WR: A dimeric creatine kinase from a sponge: implications in terms of phosphagen kinase evolution. Comp Biochem Physiol B Biochem Mol Biol. 2000 May;126(1):1-7. [10825659 ]
  10. Fredericks S, Murray JF, Bewick M, Chang R, Collinson PO, Carter ND, Holt DW: Cardiac troponin T and creatine kinase MB are not increased in exterior oblique muscle of patients with renal failure. Clin Chem. 2001 Jun;47(6):1023-30. [11375287 ]
  11. Jiao YF, Okumiya T, Saibara T, Kudo Y, Sugiura T: Erythrocyte creatine as a marker of excessive erythrocyte destruction due to hypersplenism in patients with liver cirrhosis. Clin Biochem. 2001 Jul;34(5):395-8. [11522277 ]
  12. Nicholson JK, Buckingham MJ, Sadler PJ: High resolution 1H n.m.r. studies of vertebrate blood and plasma. Biochem J. 1983 Jun 1;211(3):605-15. [6411064 ]
  13. Sheth NP, Sennett B, Berns JS: Rhabdomyolysis and acute renal failure following arthroscopic knee surgery in a college football player taking creatine supplements. Clin Nephrol. 2006 Feb;65(2):134-7. [16509464 ]
  14. Jenkins CB, Ghidini A, Spong CY, Eglinton GS, Pezzullo JC, Michejda M: Evaluation of early second trimester maternal serum creatine kinase isoenzyme BB as a marker of poor pregnancy outcome. Fetal Diagn Ther. 1997 Nov-Dec;12(6):356-9. [9475367 ]
  15. Lukaszuk JM, Robertson RJ, Arch JE, Moyna NM: Effect of a defined lacto-ovo-vegetarian diet and oral creatine monohydrate supplementation on plasma creatine concentration. J Strength Cond Res. 2005 Nov;19(4):735-40. [16287366 ]
  16. Preen DB, Dawson BT, Goodman C, Beilby J, Ching S: Comparison of erythrocyte and skeletal muscle creatine accumulation following creatine loading. Int J Sport Nutr Exerc Metab. 2005 Feb;15(1):84-93. [15902992 ]
  17. Poortmans JR, Kumps A, Duez P, Fofonka A, Carpentier A, Francaux M: Effect of oral creatine supplementation on urinary methylamine, formaldehyde, and formate. Med Sci Sports Exerc. 2005 Oct;37(10):1717-20. [16260971 ]
  18. Yoshizumi WM, Tsourounis C: Effects of creatine supplementation on renal function. J Herb Pharmacother. 2004;4(1):1-7. [15273072 ]
  19. Harris RC, Almada AL, Harris DB, Dunnett M, Hespel P: The creatine content of Creatine Serum and the change in the plasma concentration with ingestion of a single dose. J Sports Sci. 2004 Sep;22(9):851-7. [15513279 ]
  20. Mendes RR, Pires I, Oliveira A, Tirapegui J: Effects of creatine supplementation on the performance and body composition of competitive swimmers. J Nutr Biochem. 2004 Aug;15(8):473-8. [15302082 ]
  21. Burke DG, Chilibeck PD, Parise G, Tarnopolsky MA, Candow DG: Effect of alpha-lipoic acid combined with creatine monohydrate on human skeletal muscle creatine and phosphagen concentration. Int J Sport Nutr Exerc Metab. 2003 Sep;13(3):294-302. [14669930 ]
  22. Hultman E, Soderlund K, Timmons JA, Cederblad G, Greenhaff PL: Muscle creatine loading in men. J Appl Physiol (1985). 1996 Jul;81(1):232-7. [8828669 ]
  23. Dangott B, Schultz E, Mozdziak PE: Dietary creatine monohydrate supplementation increases satellite cell mitotic activity during compensatory hypertrophy. Int J Sports Med. 2000 Jan;21(1):13-6. [10683092 ]
  24. Hespel P, Op't Eijnde B, Van Leemputte M, Urso B, Greenhaff PL, Labarque V, Dymarkowski S, Van Hecke P, Richter EA: Oral creatine supplementation facilitates the rehabilitation of disuse atrophy and alters the expression of muscle myogenic factors in humans. J Physiol. 2001 Oct 15;536(Pt 2):625-33. [11600695 ]
  25. Juhn M: Popular sports supplements and ergogenic aids. Sports Med. 2003;33(12):921-39. [12974658 ]

Enzymes

General function:
Involved in guanidinoacetate N-methyltransferase activity
Specific function:
Not Available
Gene Name:
GAMT
Uniprot ID:
Q14353
Molecular weight:
26317.925
Reactions
S-Adenosylmethionine + Guanidoacetic acid → S-Adenosylhomocysteine + Creatinedetails
References
  1. Almeida LS, Vilarinho L, Darmin PS, Rosenberg EH, Martinez-Munoz C, Jakobs C, Salomons GS: A prevalent pathogenic GAMT mutation (c.59G>C) in Portugal. Mol Genet Metab. 2007 May;91(1):1-6. Epub 2007 Mar 1. [17336114 ]
  2. Kan HE, Meeuwissen E, van Asten JJ, Veltien A, Isbrandt D, Heerschap A: Creatine uptake in brain and skeletal muscle of mice lacking guanidinoacetate methyltransferase assessed by magnetic resonance spectroscopy. J Appl Physiol. 2007 Jun;102(6):2121-7. Epub 2007 Mar 8. [17347380 ]
  3. Wang L, Zhang Y, Shao M, Zhang H: Spatiotemporal expression of the creatine metabolism related genes agat, gamt and ct1 during zebrafish embryogenesis. Int J Dev Biol. 2007;51(3):247-53. [17486546 ]
General function:
Amino acid transport and metabolism
Specific function:
Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development. May be involved in the response to heart failure by elevating local creatine synthesis.
Gene Name:
GATM
Uniprot ID:
P50440
Molecular weight:
48455.01
General function:
Involved in kinase activity
Specific function:
Reversibly catalyzes the transfer of phosphate between ATP and various phosphogens (e.g. creatine phosphate). Creatine kinase isoenzymes play a central role in energy transduction in tissues with large, fluctuating energy demands, such as skeletal muscle, heart, brain and spermatozoa.
Gene Name:
CKMT2
Uniprot ID:
P17540
Molecular weight:
47504.08
Reactions
Adenosine triphosphate + Creatine → ADP + Phosphocreatinedetails
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [17016423 ]
  3. Koufen P, Ruck A, Brdiczka D, Wendt S, Wallimann T, Stark G: Free radical-induced inactivation of creatine kinase: influence on the octameric and dimeric states of the mitochondrial enzyme (Mib-CK). Biochem J. 1999 Dec 1;344 Pt 2:413-7. [10567223 ]
  4. Wyss M, James P, Schlegel J, Wallimann T: Limited proteolysis of creatine kinase. Implications for three-dimensional structure and for conformational substrates. Biochemistry. 1993 Oct 12;32(40):10727-35. [8399219 ]
  5. Stachowiak O, Dolder M, Wallimann T, Richter C: Mitochondrial creatine kinase is a prime target of peroxynitrite-induced modification and inactivation. J Biol Chem. 1998 Jul 3;273(27):16694-9. [9642223 ]
  6. Wallimann T, Wyss M, Brdiczka D, Nicolay K, Eppenberger HM: Intracellular compartmentation, structure and function of creatine kinase isoenzymes in tissues with high and fluctuating energy demands: the 'phosphocreatine circuit' for cellular energy homeostasis. Biochem J. 1992 Jan 1;281 ( Pt 1):21-40. [1731757 ]
General function:
Involved in kinase activity
Specific function:
Reversibly catalyzes the transfer of phosphate between ATP and various phosphogens (e.g. creatine phosphate). Creatine kinase isoenzymes play a central role in energy transduction in tissues with large, fluctuating energy demands, such as skeletal muscle, heart, brain and spermatozoa.
Gene Name:
CKB
Uniprot ID:
P12277
Molecular weight:
42643.95
Reactions
Adenosine triphosphate + Creatine → ADP + Phosphocreatinedetails
References
  1. Tian XF, Zhang XS, Li YH, Wang ZZ, Zhang F, Wang LM, Yao JH: Proteasome inhibition attenuates lung injury induced by intestinal ischemia reperfusion in rats. Life Sci. 2006 Oct 26;79(22):2069-76. Epub 2006 Jun 23. [16875703 ]
  2. Debrincat MA, Zhang JG, Willson TA, Silke J, Connolly LM, Simpson RJ, Alexander WS, Nicola NA, Kile BT, Hilton DJ: Ankyrin repeat and suppressors of cytokine signaling box protein asb-9 targets creatine kinase B for degradation. J Biol Chem. 2007 Feb 16;282(7):4728-37. Epub 2006 Dec 5. [17148442 ]
  3. Burklen TS, Hirschy A, Wallimann T: Brain-type creatine kinase BB-CK interacts with the Golgi Matrix Protein GM130 in early prophase. Mol Cell Biochem. 2007 Mar;297(1-2):53-64. Epub 2006 Oct 12. [17036164 ]
  4. Wallimann T, Wyss M, Brdiczka D, Nicolay K, Eppenberger HM: Intracellular compartmentation, structure and function of creatine kinase isoenzymes in tissues with high and fluctuating energy demands: the 'phosphocreatine circuit' for cellular energy homeostasis. Biochem J. 1992 Jan 1;281 ( Pt 1):21-40. [1731757 ]
General function:
Involved in kinase activity
Specific function:
Reversibly catalyzes the transfer of phosphate between ATP and various phosphogens (e.g. creatine phosphate). Creatine kinase isoenzymes play a central role in energy transduction in tissues with large, fluctuating energy demands, such as skeletal muscle, heart, brain and spermatozoa.
Gene Name:
CKMT1A
Uniprot ID:
P12532
Molecular weight:
47036.3
Reactions
Adenosine triphosphate + Creatine → ADP + Phosphocreatinedetails
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [17016423 ]
  3. Slenzka K, Appel R, Kappel T, Rahmann H: Influence of altered gravity on brain cellular energy and plasma membrane metabolism of developing lower aquatic vertebrates. Adv Space Res. 1996;17(6-7):125-8. [11538605 ]
  4. Wyss M, Schlegel J, James P, Eppenberger HM, Wallimann T: Mitochondrial creatine kinase from chicken brain. Purification, biophysical characterization, and generation of heterodimeric and heterooctameric molecules with subunits of other creatine kinase isoenzymes. J Biol Chem. 1990 Sep 15;265(26):15900-8. [2394753 ]
  5. Muhlebach SM, Wirz T, Brandle U, Perriard JC: Evolution of the creative kinases. The chicken acidic type mitochondrial creatine kinase gene as the first nonmammalian gene. J Biol Chem. 1996 May 17;271(20):11920-9. [8662608 ]
  6. Wallimann T, Wyss M, Brdiczka D, Nicolay K, Eppenberger HM: Intracellular compartmentation, structure and function of creatine kinase isoenzymes in tissues with high and fluctuating energy demands: the 'phosphocreatine circuit' for cellular energy homeostasis. Biochem J. 1992 Jan 1;281 ( Pt 1):21-40. [1731757 ]
General function:
Involved in kinase activity
Specific function:
Reversibly catalyzes the transfer of phosphate between ATP and various phosphogens (e.g. creatine phosphate). Creatine kinase isoenzymes play a central role in energy transduction in tissues with large, fluctuating energy demands, such as skeletal muscle, heart, brain and spermatozoa.
Gene Name:
CKM
Uniprot ID:
P06732
Molecular weight:
43100.91
Reactions
Adenosine triphosphate + Creatine → ADP + Phosphocreatinedetails
References
  1. Zeng L, Hu Q, Wang X, Mansoor A, Lee J, Feygin J, Zhang G, Suntharalingam P, Boozer S, Mhashilkar A, Panetta CJ, Swingen C, Deans R, From AH, Bache RJ, Verfaillie CM, Zhang J: Bioenergetic and functional consequences of bone marrow-derived multipotent progenitor cell transplantation in hearts with postinfarction left ventricular remodeling. Circulation. 2007 Apr 10;115(14):1866-75. Epub 2007 Mar 26. [17389266 ]
  2. Zhou DQ, Hu Y, Liu G, Gong L, Xi Y, Wen L: Muscle-specific creatine kinase gene polymorphism and running economy responses to an 18-week 5000-m training programme. Br J Sports Med. 2006 Dec;40(12):988-91. Epub 2006 Sep 25. [17000714 ]
  3. Wallimann T, Wyss M, Brdiczka D, Nicolay K, Eppenberger HM: Intracellular compartmentation, structure and function of creatine kinase isoenzymes in tissues with high and fluctuating energy demands: the 'phosphocreatine circuit' for cellular energy homeostasis. Biochem J. 1992 Jan 1;281 ( Pt 1):21-40. [1731757 ]
General function:
Involved in neurotransmitter:sodium symporter activity
Specific function:
Required for the uptake of creatine in muscles and brain.
Gene Name:
SLC6A8
Uniprot ID:
P48029
Molecular weight:
69483.91
References
  1. Rosenberg EH, Munoz CM, Degrauw TJ, Jakobs C, Salomons GS: Overexpression of wild-type creatine transporter (SLC6A8) restores creatine uptake in primary SLC6A8-deficient fibroblasts. J Inherit Metab Dis. 2006 Apr-Jun;29(2-3):345-6. [16763899 ]
  2. Derave W, Straumann N, Olek RA, Hespel P: Electrolysis stimulates creatine transport and transporter cell surface expression in incubated mouse skeletal muscle: potential role of ROS. Am J Physiol Endocrinol Metab. 2006 Dec;291(6):E1250-7. Epub 2006 Jul 18. [16849631 ]
  3. Lunardi G, Parodi A, Perasso L, Pohvozcheva AV, Scarrone S, Adriano E, Florio T, Gandolfo C, Cupello A, Burov SV, Balestrino M: The creatine transporter mediates the uptake of creatine by brain tissue, but not the uptake of two creatine-derived compounds. Neuroscience. 2006 Nov 3;142(4):991-7. Epub 2006 Sep 1. [16949212 ]
  4. Campistol J, Arias-Dimas A, Poo P, Pineda M, Hoffman M, Vilaseca MA, Artuch R, Ribes A: [Cerebral creatine transporter deficiency: an infradiagnosed neurometabolic disease]. Rev Neurol. 2007 Mar 16-31;44(6):343-7. [17385170 ]
  5. Wang L, Zhang Y, Shao M, Zhang H: Spatiotemporal expression of the creatine metabolism related genes agat, gamt and ct1 during zebrafish embryogenesis. Int J Dev Biol. 2007;51(3):247-53. [17486546 ]
General function:
Involved in DNA binding
Specific function:
Acts as a transcriptional regulator of muscle creatine kinase (MCK) and so has a role in the establishment of diverse mesodermal muscle types. The protein binds to an A/T-rich element in the muscle creatine enhancer
Gene Name:
PRRX1
Uniprot ID:
P54821
Molecular weight:
27296.2
General function:
Involved in transcription regulator activity
Specific function:
ID (inhibitor of DNA binding) HLH proteins lack a basic DNA-binding domain but are able to form heterodimers with other HLH proteins, thereby inhibiting DNA binding. ID-3 inhibits the binding of E2A-containing protein complexes to muscle creatine kinase E-box enhancer. May inhibit other transcription factors
Gene Name:
ID3
Uniprot ID:
Q02535
Molecular weight:
12968.8

Transporters

General function:
Involved in ion transmembrane transporter activity
Specific function:
Sodium-ion dependent, high affinity carnitine transporter. Involved in the active cellular uptake of carnitine. Transports one sodium ion with one molecule of carnitine. Also transports organic cations such as tetraethylammonium (TEA) without the involvement of sodium. Also relative uptake activity ratio of carnitine to TEA is 11.3
Gene Name:
SLC22A5
Uniprot ID:
O76082
Molecular weight:
62751.1
References
  1. Ohashi R, Tamai I, Yabuuchi H, Nezu JI, Oku A, Sai Y, Shimane M, Tsuji A: Na(+)-dependent carnitine transport by organic cation transporter (OCTN2): its pharmacological and toxicological relevance. J Pharmacol Exp Ther. 1999 Nov;291(2):778-84. [10525100 ]