MiMeDB: Showing metabocard for Guanidoacetic acid (MMDBc0000496)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2020-12-10 18:39:05 UTC

Update Date

2024-09-29 15:09:28 UTC

Metabolite ID

MMDBc0000496

Metabolite Identification

Common Name

Guanidoacetic acid

Description

Guanidoacetic 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

MOL 3D MOL SDF 3D SDF PDB 3D PDB SMILES InChI

Synonyms

Value	Source
Glycocyamine	ChEBI
Guanidinoacetate	ChEBI
N-(Carbamimidoyl)glycine	ChEBI
N-[Amino(imino)methyl]glycine	ChEBI
N-Amidinoglycine	ChEBI
Guanidinoacetic acid	Kegg
Guanidoacetate	Generator
(Carboxymethyl)-guanidine	HMDB
2-[[Amino(imino)methyl]amino]acetate	HMDB
2-[[Amino(imino)methyl]amino]acetic acid	HMDB
a-Guanidinoacetate	HMDB
a-Guanidinoacetic acid	HMDB
alpha-Guanidinoacetate	HMDB
alpha-Guanidinoacetic acid	HMDB
b-Guanidinoacetate	HMDB
b-Guanidinoacetic acid	HMDB
beta-Guanidinoacetate	HMDB
beta-Guanidinoacetic acid	HMDB
Betacyamine	HMDB
Betasyamine	HMDB
Guanidineacetate	HMDB
Guanidineacetic acid	HMDB
Guanidylacetate	HMDB
Guanidylacetic acid	HMDB
Guanyl glycine	HMDB
N-Amidino-glycine	HMDB
[(Aminoiminomethyl)amino]-acetate	HMDB
[(Aminoiminomethyl)amino]-acetic acid	HMDB
Glycocyamine, 2-(14)C-labeled	HMDB
Glycocyamine, ion (1-)	HMDB
Glycocyamine monohydrochloride	HMDB

Molecular Formula

C₃H₇N₃O₂

Average Mass

117.1066

Monoisotopic Mass

117.053826483

IUPAC Name

2-carbamimidamidoacetic acid

Traditional Name

glycocyamine

CAS Registry Number

Not Available

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

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.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Amino acids, peptides, and analogues

Direct Parent

Alpha 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 Framework

Aliphatic acyclic compounds

External Descriptors

glycine derivative (CHEBI:16344 )
N-amidino-amino acid (CHEBI:16344 )

Functional Ontology

Not Available

Physical Properties

State

Expected Solid

Predicted Properties

Property	Value	Source
Water Solubility	4.19 g/L	ALOGPS
logP	-1.8	ALOGPS
logP	-3.1	ChemAxon
logS	-1.4	ALOGPS
pKa (Strongest Acidic)	3.37	ChemAxon
pKa (Strongest Basic)	12.24	ChemAxon
Physiological Charge	0	ChemAxon
Hydrogen Acceptor Count	5	ChemAxon
Hydrogen Donor Count	4	ChemAxon
Polar Surface Area	99.2 Å²	ChemAxon
Rotatable Bond Count	2	ChemAxon
Refractivity	36.72 m³·mol⁻¹	ChemAxon
Polarizability	10.54 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Spectra

Spectrum Type	Description	Splash Key	Deposition Date	View
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-00di-0901000000-4a89fe463d29379e6339	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-00di-0911000000-542baca9dcca73225bf0	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-006t-0911200000-bb7ea068f193f39c283b	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-00di-0901000000-4a89fe463d29379e6339	2018-05-18	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-00di-0911000000-542baca9dcca73225bf0	2018-05-18	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-006t-0911200000-bb7ea068f193f39c283b	2018-05-18	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-0006-9100000000-c703c631a81e400681bd	2017-08-28	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positive	splash10-00dl-9700000000-1dae820000c4f9ec1662	2017-10-06	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_4) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_4) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)	splash10-016r-7900000000-4777d20e1ab29262427e	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)	splash10-00dl-9000000000-a1b5501bf0f7a9b0489d	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)	splash10-0006-9100000000-b61a48cf749b57a2c77e	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positive	splash10-0gk9-7900000000-5ba298286886c1fabdf5	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative	splash10-00di-9100000000-a5b77a913821d637210f	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF , negative	splash10-00di-9100000000-a5b77a913821d637210f	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF , positive	splash10-0gk9-7900000000-5ba298286886c1fabdf5	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - , positive	splash10-014i-6900000000-4d655a894c702bdfdb59	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 40V, Negative	splash10-0006-9000000000-23d5c2795ed49f043127	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 20V, Negative	splash10-00di-9000000000-ae360cd8919c8fbce3ee	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Negative	splash10-014i-0900000000-93406e2cdb79770abc7d	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Negative	splash10-00di-9000000000-c6bd00733ea6fa3f6e5d	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 20V, Positive	splash10-0ab9-9000000000-2667e0007de6a723415e	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Positive	splash10-0100-9400000000-c5561d5ccaf260c048ec	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Positive	splash10-0g29-9400000000-421d076c4b6868ca69fa	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 20V, Negative	splash10-014i-0900000000-53db4bf2729a2990a938	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 20V, Positive	splash10-05fu-9000000000-2cad7322e574eb992175	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 35V, Positive	splash10-01di-9800000000-967331a78fe4a7917408	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 20V, Positive	splash10-00dl-9000000000-7b281fae734baaa2695d	2021-09-20	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-014i-4900000000-1b831140a3071dd98e0c	2017-07-25	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-00di-9300000000-b27aea734be08f5d2ddc	2017-07-25	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-00dl-9000000000-dea2f28faa5430d7e56f	2017-07-25	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-00xr-9500000000-1ba345c7064e9cb70f9c	2017-07-26	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-00di-9000000000-6864d4355d3462fbc69f	2017-07-26	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-0006-9000000000-1c36299ab8746bbf67a5	2017-07-26	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, H₂O, experimental)		2012-12-04	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 125 MHz, H₂O, experimental)		2012-12-04	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-29	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-29	View Spectrum
2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 400 MHz, H₂O, experimental)		2012-12-04	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm

Biospecimen Locations

Blood
Brain
Cellular Cytoplasm
Cerebrospinal Fluid (CSF)
Feces
Kidney
Liver
Placenta
Urine

Tissue Locations

Brain
Kidney
Liver
Placenta

Associated OMIM IDs

114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)

Human Proteins and Enzymes

Protein ID	Protein Name	Protein Type	Uniprot ID	Species
MMDBp0192092	Guanidinoacetate N-methyltransferase	Unknown	Q14353	Homo sapiens
MMDBp0192122	Glycine amidinotransferase, mitochondrial	Enzyme	P50440	Homo sapiens

Human Pathways

Pathways

Name	SMPDB/PathBank

Metabolic Reactions

Reactions

Reaction ID	Precursor	Product	Enzyme	Reaction Type	Data Source	Reference
MMDBr0001263	S-Adenosylmethionine	S-Adenosylhomocysteine	guanidinoacetate N-methyltransferase		VMH	2001229
MMDBr0001350	L-Arginine	Guanidoacetic acid	glycine amidinotransferase (L-arginine:glycine amidinotransferase)		VMH	8547310

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Evidence Type	Host and Biospecimen	Data Source	Reference
Uremia		Association	Human Blood	HMDB	22626821
Uremia	Increased	Association	Human Blood	HMDB	12675874
Eosinophilic esophagitis		Association	Human Urine	HMDB	Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)

Microbial Sources

Superkingdom/Kingdom	Phylum	Total species	Hosts and Body Sites	Microbial Links
Bacteria	Proteobacteria	1	Human	Indirect association between microbiota composition and metabolites measured in host biospecimen

Exposure Sources

Source Type	Source Sub-type	Species	Data Source	Reference
Plant	Shoot	Malus pumila	FooDB	31437929
Plant	Shoot	Eriobotrya japonica	FooDB	31437929

Host Biospecimen and Location

Host and Biospecimen	Status	Mean	Std	Min	Max	Units	Age	Sex	Health Condition	Data Source	Reference
Human Feces	Detected but not Quantified									HMDB
Human Blood	Detected and Quantified	1.1				uM	Children (1-13 years old)	Male	Cerebral creatine deficiency syndrome 1	HMDB	11326334
Human Urine	Detected and Quantified	74				umol/mmol creatinine	Children (1-13 years old)	Male	Cerebral creatine deficiency syndrome 1	HMDB	11326334
Human Cerebrospinal fluid (csf)	Detected and Quantified			11.0	12.4	uM	Adult (>18 years old)	Female	Cerebral creatine deficiency syndrome 2	HMDB	15651030
Human Blood	Detected and Quantified			17.3	27.0	uM	Adult (>18 years old)	Female	Cerebral creatine deficiency syndrome 2	HMDB	15651030
Human Urine	Detected and Quantified			614	1624	umol/mmol creatinine	Adult (>18 years old)	Female	Cerebral creatine deficiency syndrome 2	HMDB	15651030
Human Blood	Detected and Quantified			0.2	0.3	uM	Children (1-13 years old)	Female	Cerebral creatine deficiency syndrome 3	HMDB	23770102
Human Urine	Detected and Quantified			0.9	2.6	umol/mmol creatinine	Children (1-13 years old)	Female	Cerebral creatine deficiency syndrome 3	HMDB	23770102
Human Blood	Detected and Quantified	2.4	0.7			uM	Adult (>18 years old)	Both	Chronic renal failure	HMDB	7296840
Human Blood	Detected and Quantified	1.88	0.30			uM	Adult (>18 years old)	Both	uremia	HMDB	22626821
Human Blood	Detected and Quantified	3.280	1.230			uM	Adult (>18 years old)	Both	Uremia	HMDB	12675874
Human Urine	Detected and Quantified	127.26				umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	24909877
Human Urine	Detected and Quantified	<150				umol/mmol creatinine	Adult (>18 years old)	Female	Normal	HMDB	15651030
Human Cerebrospinal fluid (csf)	Detected and Quantified	0.05	0.03			uM	Adult (>18 years old)	Both	Normal	HMDB	3433275
Human Cerebrospinal fluid (csf)	Detected and Quantified			0.068	0.114	uM	Adult (>18 years old)	Female	Normal	HMDB	15651030
Human Blood	Detected and Quantified			0.35	8.0	uM	Children (1-13 years old)	Not Specified	Normal	HMDB	23770102
Human Blood	Detected and Quantified			0.7	1.4	uM	Children (1-13 years old)	Both	Normal	HMDB	11326334
Human Blood	Detected and Quantified			0.7	3.5	uM	Adult (>18 years old)	Both	Normal	HMDB	11326334
Human Blood	Detected and Quantified			1.0	3.5	uM	Children (1-13 years old)	Not Specified	Normal	HMDB	23770102
Human Blood	Detected and Quantified	1.22	0.40			uM	Adult (>18 years old)	Both	Normal	HMDB	3433275
Human Blood	Detected and Quantified			1.3	3.3	uM	Adult (>18 years old)	Female	Normal	HMDB	15651030
Human Blood	Detected and Quantified	1.90	0.68			uM	Adult (>18 years old)	Both	Normal	HMDB	22626821
Human Blood	Detected and Quantified	1.900	0.680			uM	Adult (>18 years old)	Both	Normal	HMDB	12675874
Human Urine	Detected and Quantified			10	99	umol/mmol creatinine	Children (1-13 years old)	Both	Normal	HMDB	11326334
Human Urine	Detected and Quantified	13.3		5.2	21.5	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	18818040
Human Blood	Detected and Quantified	16.8		0.81	32.9	uM	Adult (>18 years old)	Both	Normal	HMDB	18818040
Human Blood	Detected and Quantified	2.0		0.0	5.0	uM	Adult (>18 years old)	Both	Normal	HMDB	12661026
Human Blood	Detected and Quantified	2.8	0.9			uM	Adult (>18 years old)	Both	Normal	HMDB	7296840
Human Urine	Detected and Quantified			3	78	umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	23770102
Human Blood	Detected and Quantified	3.08		0.87	3.64	uM	Adult (>18 years old)	Both	Normal	HMDB	16736098
Human Urine	Detected and Quantified			35	259	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	24909877
Human Urine	Detected and Quantified			4.0	220	umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	23770102
Human Urine	Detected and Quantified	41.8		10.6	97.3	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected and Quantified	74.0		18.0	130.0	umol/mmol creatinine	Children (1-13 years old)	Both	Normal	HMDB	15369749
Human Urine	Detected and Quantified	88.5		18.0	159.0	umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	15369749
Human Urine	Detected and Quantified	89.0		11.0	124.0	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	12661026
Human Urine	Detected and Quantified			93	190	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	31506554
Human Urine	Detected and Quantified	55.592	42.373			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	115.283	130.147			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified			16	228	umol/mmol creatinine	Children (1 - 18 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Urine	Detected and Quantified			12.81	38.44	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	David F. Putnam Composition and Concentrative Properties of Human Urine. NASA Contractor Report. July 1971
Human Urine	Detected and Quantified			7.69	21.36	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	David F. Putnam Composition and Concentrative Properties of Human Urine. NASA Contractor Report. July 1971
Human Urine	Detected and Quantified	15.4	10.0			umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Blood	Detected and Quantified	19.0		3.0	37.0	uM	Adult (>18 years old)	Not Specified	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Cellular cytoplasm	Detected and Quantified	2.3	0.9			uM	Adult (>18 years old)	Not Specified	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Urine	Detected and Quantified	9.33				umol/mmol creatinine	Adult (>18 years old)	Male	Normal	HMDB	Shaykhutdinov RA, MacInnis GD, Dowlatabadi R, Weljie AM, Vogel HJ. Quantitative analysis of metabolite concentrations in human urine samples using 13C{1H} NMR spectroscopy. Metabolomics. 2009
Human Brain	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Kidney	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Liver	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Placenta	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	21389975
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	22024767
Human Feces	Detected but not Quantified						Children (6 - 18 years old)	Not Specified	Normal	HMDB	27609529
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	27543620
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	29808030
Human Blood	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	32966057

External Links

HMDB ID

HMDB0000128

DrugBank ID

DB02751

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB005417

KNApSAcK ID

Not Available

Chemspider ID

KEGG Compound ID

BioCyc ID

BiGG ID

Wikipedia Link

METLIN ID

PubChem Compound

PDB ID

Not Available

ChEBI ID

16344

Food Biomarker Ontology

Not Available

References

Synthesis Reference

Not Available

General References

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. doi: 10.1620/tjem.153.197. [PubMed:3433275 ]
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 ]
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. doi: 10.1203/00006450-199409000-00023. [PubMed:7808840 ]
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. doi: 10.1016/0167-4781(95)00184-0. [PubMed:8547310 ]
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 ]
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. doi: 10.1006/abio.1997.2343. [PubMed:9367508 ]
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. doi: 10.1006/mgme.2001.3257. [PubMed:11749046 ]
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. doi: 10.1002/rcm.966. [PubMed:12661026 ]
Schulze A: Creatine deficiency syndromes. Mol Cell Biochem. 2003 Feb;244(1-2):143-50. [PubMed:12701824 ]
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. doi: 10.1093/hmg/ddh112. Epub 2004 Mar 17. [PubMed:15028668 ]
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. doi: 10.1016/j.ymgme.2004.04.005. [PubMed:15234333 ]
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. doi: 10.1016/j.cccn.2004.05.013. [PubMed:15369749 ]
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. doi: 10.1016/j.ymgme.2005.10.013. Epub 2005 Dec 15. [PubMed:16343968 ]
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. doi: 10.1212/01.wnl.0000234852.43688.bf. Epub 2006 Jul 19. [PubMed:16855203 ]
Duranton F, Cohen G, De Smet R, Rodriguez M, Jankowski J, Vanholder R, Argiles A: Normal and pathologic concentrations of uremic toxins. J Am Soc Nephrol. 2012 Jul;23(7):1258-70. doi: 10.1681/ASN.2011121175. Epub 2012 May 24. [PubMed:22626821 ]
Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

Showing metabocard for Guanidoacetic acid (MMDBc0000496)

MOL for #<Metabolite:0x0000559d0478cac0>

3D MOL for #<Metabolite:0x0000559d0478cac0>

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3D-SDF for #<Metabolite:0x0000559d0478cac0>

PDB for #<Metabolite:0x0000559d0478cac0>

3D PDB for #<Metabolite:0x0000559d0478cac0>

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INCHI for #<Metabolite:0x0000559d0478cac0>

Structure for #<Metabolite:0x0000559d0478cac0>

3D Structure for #<Metabolite:0x0000559d0478cac0>