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Showing NP-Card for Guanidoacetic acid (NP0001279)

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Record Information
Version1.0
Created at2005-11-16 15:48:42 UTC
Updated at2021-07-01 14:27:14 UTC
NP-MRD IDNP0001279
Secondary Accession NumbersNone
Natural Product Identification
Common NameGuanidoacetic acid
DescriptionGuanidoacetic acid (GAA), also known as guanidinoacetate or glycocyamine, 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. Guanidinoacetic acid was first prepared in 1861 by Adolph Strecker by reaction of cyanamide with glycine in aqueous solution. Manufactured guanidinoacetic acid is primarily used a feed additive approved by EFSA in poultry farming (for fattening), and pigs for fattening. Guanidoacetic acid exists naturally in all vertebrates. It is formed primarily in the kidneys by transferring the guanidine group of L-arginine to the amino acid glycine via the enzyme known as L-Arg:Gly-amidinotransferase (AGAT). In a further step, guanidinoacetate is methylated to generate creatine using S-adenosyl methionine (as the methyl donor) via the enzyme known as guanidinoacetate N-methyltransferase (GAMT). The resulting creatine is released into the bloodstream. Elevated levels of guanidoacetic acid are a characteristic of an inborn metabolic disorder known as Guanidinoacetate Methyltransferase (GAMT) Deficiency. GAMT converts guanidinoacetate to creatine and deficiency of this enzyme results in creatine depletion and accumulation of guanidinoacetate The disorder is transmitted in an autosomal recessive fashion and is localized to mutations on chromosome 19p13.3. GAMT deficiency is characterized by developmental arrest, medication-resistant epilepsy (myoclonic, generalized tonic-clonic, partial complex, atonic), severe speech impairment, progressive dystonia, dyskinesias, hypotonia, ataxia, and autistic-like behavior.
Structure
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MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for NP0001279 (Guanidoacetic acid)


  Mrv1652305271900142D          

  8  7  0  0  0  0            999 V2000
   -0.1914    0.7145    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
    0.5230    1.1270    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.5230    1.9520    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
    1.2375    0.7145    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
    1.9520    1.1270    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.6664    0.7145    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.3809    1.1270    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.6664   -0.1105    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  2  3  2  0  0  0  0
  2  4  1  0  0  0  0
  4  5  1  0  0  0  0
  5  6  1  0  0  0  0
  6  7  1  0  0  0  0
  6  8  2  0  0  0  0
M  END
Download

3D MOL for NP0001279 (Guanidoacetic acid)

HMDB0000128
     RDKit          3D
Guanidoacetic acid
 15 14  0  0  0  0  0  0  0  0999 V2000
    2.0620   -1.1972   -0.4757 N   0  0  0  0  0  0  0  0  0  0  0  0
    1.4586   -0.1646   -0.0219 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.8564    1.1153   -0.4704 N   0  0  0  0  0  0  0  0  0  0  0  0
    0.4159   -0.2839    0.9085 N   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9169    0.2128    0.7166 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.6521   -0.5078   -0.3249 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0876   -1.4472   -0.9314 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.9520   -0.1768   -0.6539 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.8450   -2.1749   -0.2098 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.2750    1.7707    0.2204 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.7468    1.4305   -1.4720 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.6370   -0.7748    1.8075 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4673    0.1169    1.6730 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9238    1.3269    0.5147 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.2969    0.7540   -0.7478 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  2  3
  2  3  1  0
  2  4  1  0
  4  5  1  0
  5  6  1  0
  6  7  2  0
  6  8  1  0
  1  9  1  0
  3 10  1  0
  3 11  1  0
  4 12  1  0
  5 13  1  0
  5 14  1  0
  8 15  1  0
M  END
Download

3D SDF for NP0001279 (Guanidoacetic acid)


  Mrv1652305271900142D          

  8  7  0  0  0  0            999 V2000
   -0.1914    0.7145    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
    0.5230    1.1270    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.5230    1.9520    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
    1.2375    0.7145    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
    1.9520    1.1270    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.6664    0.7145    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.3809    1.1270    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.6664   -0.1105    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  2  3  2  0  0  0  0
  2  4  1  0  0  0  0
  4  5  1  0  0  0  0
  5  6  1  0  0  0  0
  6  7  1  0  0  0  0
  6  8  2  0  0  0  0
M  END
> <DATABASE_ID>
NP0001279

> <DATABASE_NAME>
NP-MRD

> <SMILES>
NC(=N)NCC(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C3H7N3O2/c4-3(5)6-1-2(7)8/h1H2,(H,7,8)(H4,4,5,6)

> <INCHI_KEY>
BPMFZUMJYQTVII-UHFFFAOYSA-N

> <FORMULA>
C3H7N3O2

> <MOLECULAR_WEIGHT>
117.1066

> <EXACT_MASS>
117.053826483

> <JCHEM_ACCEPTOR_COUNT>
5

> <JCHEM_ATOM_COUNT>
15

> <JCHEM_AVERAGE_POLARIZABILITY>
10.53697099662325

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
4

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
2-carbamimidamidoacetic acid

> <ALOGPS_LOGP>
-1.76

> <JCHEM_LOGP>
-3.1262055894355907

> <ALOGPS_LOGS>
-1.45

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
0

> <JCHEM_PKA_STRONGEST_ACIDIC>
3.3686966251675314

> <JCHEM_PKA_STRONGEST_BASIC>
12.241335630709775

> <JCHEM_POLAR_SURFACE_AREA>
99.20000000000002

> <JCHEM_REFRACTIVITY>
36.718

> <JCHEM_ROTATABLE_BOND_COUNT>
2

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
4.19e+00 g/l

> <JCHEM_TRADITIONAL_IUPAC>
glycocyamine

> <JCHEM_VEBER_RULE>
0

$$$$
Download

3D-SDF for NP0001279 (Guanidoacetic acid)

HMDB0000128
     RDKit          3D
Guanidoacetic acid
 15 14  0  0  0  0  0  0  0  0999 V2000
    2.0620   -1.1972   -0.4757 N   0  0  0  0  0  0  0  0  0  0  0  0
    1.4586   -0.1646   -0.0219 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.8564    1.1153   -0.4704 N   0  0  0  0  0  0  0  0  0  0  0  0
    0.4159   -0.2839    0.9085 N   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9169    0.2128    0.7166 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.6521   -0.5078   -0.3249 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0876   -1.4472   -0.9314 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.9520   -0.1768   -0.6539 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.8450   -2.1749   -0.2098 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.2750    1.7707    0.2204 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.7468    1.4305   -1.4720 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.6370   -0.7748    1.8075 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4673    0.1169    1.6730 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9238    1.3269    0.5147 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.2969    0.7540   -0.7478 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  2  3
  2  3  1  0
  2  4  1  0
  4  5  1  0
  5  6  1  0
  6  7  2  0
  6  8  1  0
  1  9  1  0
  3 10  1  0
  3 11  1  0
  4 12  1  0
  5 13  1  0
  5 14  1  0
  8 15  1  0
M  END
Download

PDB for NP0001279 (Guanidoacetic acid)

HEADER    PROTEIN                                 27-MAY-19   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   27-MAY-19         0                                  
HETATM    1  N   UNK     0      -0.357   1.334   0.000  0.00  0.00           N+0
HETATM    2  C   UNK     0       0.976   2.104   0.000  0.00  0.00           C+0
HETATM    3  N   UNK     0       0.976   3.644   0.000  0.00  0.00           N+0
HETATM    4  N   UNK     0       2.310   1.334   0.000  0.00  0.00           N+0
HETATM    5  C   UNK     0       3.644   2.104   0.000  0.00  0.00           C+0
HETATM    6  C   UNK     0       4.977   1.334   0.000  0.00  0.00           C+0
HETATM    7  O   UNK     0       6.311   2.104   0.000  0.00  0.00           O+0
HETATM    8  O   UNK     0       4.977  -0.206   0.000  0.00  0.00           O+0
CONECT    1    2                                                            
CONECT    2    1    3    4                                                  
CONECT    3    2                                                            
CONECT    4    2    5                                                       
CONECT    5    4    6                                                       
CONECT    6    5    7    8                                                  
CONECT    7    6                                                            
CONECT    8    6                                                            
MASTER        0    0    0    0    0    0    0    0    8    0   14    0
END
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3D PDB for NP0001279 (Guanidoacetic acid)

COMPND    HMDB0000128
HETATM    1  N1  UNL     1       2.062  -1.197  -0.476  1.00  0.00           N  
HETATM    2  C1  UNL     1       1.459  -0.165  -0.022  1.00  0.00           C  
HETATM    3  N2  UNL     1       1.856   1.115  -0.470  1.00  0.00           N  
HETATM    4  N3  UNL     1       0.416  -0.284   0.909  1.00  0.00           N  
HETATM    5  C2  UNL     1      -0.917   0.213   0.717  1.00  0.00           C  
HETATM    6  C3  UNL     1      -1.652  -0.508  -0.325  1.00  0.00           C  
HETATM    7  O1  UNL     1      -1.088  -1.447  -0.931  1.00  0.00           O  
HETATM    8  O2  UNL     1      -2.952  -0.177  -0.654  1.00  0.00           O  
HETATM    9  H1  UNL     1       1.845  -2.175  -0.210  1.00  0.00           H  
HETATM   10  H2  UNL     1       2.275   1.771   0.220  1.00  0.00           H  
HETATM   11  H3  UNL     1       1.747   1.431  -1.472  1.00  0.00           H  
HETATM   12  H4  UNL     1       0.637  -0.775   1.808  1.00  0.00           H  
HETATM   13  H5  UNL     1      -1.467   0.117   1.673  1.00  0.00           H  
HETATM   14  H6  UNL     1      -0.924   1.327   0.515  1.00  0.00           H  
HETATM   15  H7  UNL     1      -3.297   0.754  -0.748  1.00  0.00           H  
CONECT    1    2    2    9
CONECT    2    3    4
CONECT    3   10   11
CONECT    4    5   12
CONECT    5    6   13   14
CONECT    6    7    7    8
CONECT    8   15
END
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SMILES for NP0001279 (Guanidoacetic acid)

NC(=N)NCC(O)=O
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INCHI for NP0001279 (Guanidoacetic acid)

InChI=1S/C3H7N3O2/c4-3(5)6-1-2(7)8/h1H2,(H,7,8)(H4,4,5,6)
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View in JSmol
Synonyms
ValueSource
GlycocyamineChEBI
GuanidinoacetateChEBI
N-(Carbamimidoyl)glycineChEBI
N-[Amino(imino)methyl]glycineChEBI
N-AmidinoglycineChEBI
Guanidinoacetic acidKegg
GuanidoacetateGenerator
(Carboxymethyl)-guanidineHMDB
2-[[Amino(imino)methyl]amino]acetateHMDB
2-[[Amino(imino)methyl]amino]acetic acidHMDB
a-GuanidinoacetateHMDB
a-Guanidinoacetic acidHMDB
alpha-GuanidinoacetateHMDB
alpha-Guanidinoacetic acidHMDB
b-GuanidinoacetateHMDB
b-Guanidinoacetic acidHMDB
beta-GuanidinoacetateHMDB
beta-Guanidinoacetic acidHMDB
BetacyamineHMDB
BetasyamineHMDB
GuanidineacetateHMDB
Guanidineacetic acidHMDB
GuanidylacetateHMDB
Guanidylacetic acidHMDB
Guanyl glycineHMDB
N-Amidino-glycineHMDB
[(Aminoiminomethyl)amino]-acetateHMDB
[(Aminoiminomethyl)amino]-acetic acidHMDB
Glycocyamine, 2-(14)C-labeledHMDB
Glycocyamine, ion (1-)HMDB
Glycocyamine monohydrochlorideHMDB
Chemical FormulaC3H7N3O2
Average Mass117.1066 Da
Monoisotopic Mass117.05383 Da
IUPAC Name2-carbamimidamidoacetic acid
Traditional Nameglycocyamine
CAS Registry Number352-97-6
SMILES
NC(=N)NCC(O)=O
InChI Identifier
InChI=1S/C3H7N3O2/c4-3(5)6-1-2(7)8/h1H2,(H,7,8)(H4,4,5,6)
InChI KeyBPMFZUMJYQTVII-UHFFFAOYSA-N
Experimental Spectra
Spectrum TypeDescriptionDepositor EmailDepositor OrganizationDepositorDeposition DateView
1D NMR1H NMR Spectrum (1D, 500 MHz, H2O, experimental)Wishart LabWishart LabDavid Wishart2021-06-20View Spectrum
2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 400 MHz, H2O, experimental)Wishart LabWishart LabDavid Wishart2021-06-20View Spectrum
Predicted Spectra
Not Available
Chemical Shift Submissions
Not Available
Species
Species of Origin
Species NameSourceReference
Anabaena bergiiKNApSAcK Database
Chrysosporum ovalisporumBacteria
Citrus unshiuLOTUS Database
Cylindrospermopsis raciborskii-
Hippospongia communisLOTUS Database
Homo sapiensLOTUS Database
Mus musculusLOTUS Database
Raphidiopsis curvata-
Umezakia natans-
Species Where Detected
Species NameSourceReference
Aphanizomenon flos-aquaeKNApSAcK Database
Aphanizomenon ovalisporumKNApSAcK Database
Homo sapiens (Urine)KNApSAcK Database
Chemical Taxonomy
Description 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
  • Guanidine
  • Carboximidamide
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Organic nitrogen compound
  • Organic oxygen compound
  • Organopnictogen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Imine
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water Solubility3.6 mg/mL at 15 °CNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility4.19 g/LALOGPS
logP-1.8ALOGPS
logP-3.1ChemAxon
logS-1.4ALOGPS
pKa (Strongest Acidic)3.37ChemAxon
pKa (Strongest Basic)12.24ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count4ChemAxon
Polar Surface Area99.2 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity36.72 m³·mol⁻¹ChemAxon
Polarizability10.54 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
HMDB IDHMDB0000128
DrugBank IDDB02751
Phenol Explorer Compound IDNot Available
FoodDB IDFDB005417
KNApSAcK IDC00051746
Chemspider ID743
KEGG Compound IDC00581
BioCyc IDGUANIDOACETIC_ACID
BiGG ID1594816
Wikipedia LinkGlycocyamine
METLIN ID5163
PubChem Compound763
PDB IDNot Available
ChEBI ID16344
Good Scents IDrw1248881
References
General References
  1. Isbrandt D, von Figura K: Cloning and sequence analysis of human guanidinoacetate N-methyltransferase cDNA. Biochim Biophys Acta. 1995 Dec 27;1264(3):265-7. [PubMed:8547310 ]
  2. Stockler S, Isbrandt D, Hanefeld F, Schmidt B, von Figura K: Guanidinoacetate methyltransferase deficiency: the first inborn error of creatine metabolism in man. Am J Hum Genet. 1996 May;58(5):914-22. [PubMed:8651275 ]
  3. 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. [PubMed:16855203 ]
  4. Schulze A: Creatine deficiency syndromes. Mol Cell Biochem. 2003 Feb;244(1-2):143-50. [PubMed:12701824 ]
  5. Ensenauer R, Thiel T, Schwab KO, Tacke U, Stockler-Ipsiroglu S, Schulze A, Hennig J, Lehnert W: Guanidinoacetate methyltransferase deficiency: differences of creatine uptake in human brain and muscle. Mol Genet Metab. 2004 Jul;82(3):208-13. [PubMed:15234333 ]
  6. Yasuda M, Sugahara K, Zhang J, Ageta T, Nakayama K, Shuin T, Kodama H: Simultaneous determination of creatinine, creatine, and guanidinoacetic acid in human serum and urine using liquid chromatography-atmospheric pressure chemical ionization mass spectrometry. Anal Biochem. 1997 Nov 15;253(2):231-5. [PubMed:9367508 ]
  7. Sijens PE, Reijngoud DJ, Soorani-Lunsing RJ, Oudkerk M, van Spronsen FJ: Cerebral 1H MR spectroscopy showing elevation of brain guanidinoacetate in argininosuccinate lyase deficiency. Mol Genet Metab. 2006 May;88(1):100-2. Epub 2005 Dec 15. [PubMed:16343968 ]
  8. Valongo C, Cardoso ML, Domingues P, Almeida L, Verhoeven N, Salomons G, Jakobs C, Vilarinho L: Age related reference values for urine creatine and guanidinoacetic acid concentration in children and adolescents by gas chromatography-mass spectrometry. Clin Chim Acta. 2004 Oct;348(1-2):155-61. [PubMed:15369749 ]
  9. Fingerhut R: Stable isotope dilution method for the determination of guanidinoacetic acid by gas chromatography/mass spectrometry. Rapid Commun Mass Spectrom. 2003;17(7):717-22. [PubMed:12661026 ]
  10. Schmidt A, Marescau B, Boehm EA, Renema WK, Peco R, Das A, Steinfeld R, Chan S, Wallis J, Davidoff M, Ullrich K, Waldschutz R, Heerschap A, De Deyn PP, Neubauer S, Isbrandt D: Severely altered guanidino compound levels, disturbed body weight homeostasis and impaired fertility in a mouse model of guanidinoacetate N-methyltransferase (GAMT) deficiency. Hum Mol Genet. 2004 May 1;13(9):905-21. Epub 2004 Mar 17. [PubMed:15028668 ]
  11. Shirokane Y, Utsushikawa M, Nakajima M: A new enzymic determination of guanidinoacetic acid in urine. Clin Chem. 1987 Mar;33(3):394-7. [PubMed:3815805 ]
  12. Mizutani N, Hayakawa C, Ohya Y, Watanabe K, Watanabe Y, Mori A: Guanidino compounds in hyperargininemia. Tohoku J Exp Med. 1987 Nov;153(3):197-205. [PubMed:3433275 ]
  13. Schulze A, Ebinger F, Rating D, Mayatepek E: Improving treatment of guanidinoacetate methyltransferase deficiency: reduction of guanidinoacetic acid in body fluids by arginine restriction and ornithine supplementation. Mol Genet Metab. 2001 Dec;74(4):413-9. [PubMed:11749046 ]
  14. Stockler S, Holzbach U, Hanefeld F, Marquardt I, Helms G, Requart M, Hanicke W, Frahm J: Creatine deficiency in the brain: a new, treatable inborn error of metabolism. Pediatr Res. 1994 Sep;36(3):409-13. [PubMed:7808840 ]