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Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2023-05-30 20:55:54 UTC

HMDB ID

HMDB0000128

Secondary Accession Numbers

HMDB00128

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 SDF PDB SMILES InChI

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Not Available

Chemical Formula

C₃H₇N₃O₂

Average Molecular Weight

117.1066

Monoisotopic Molecular Weight

117.053826483

IUPAC Name

Not Available

Traditional Name

Not Available

CAS Registry Number

352-97-6

SMILES

Not Available

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

Classification

Not classified

Ontology

Physiological effect

Not Available

Disposition

Biological location

Non-excretory biofluid

Biofluid or Excreta

Blood (PMID: 12661026)
Cerebrospinal Fluid (CSF) (PMID: 3433275)
Cellular Cytoplasm (HMDB: HMDB0000128)

Organ

Excreta

Biofluid or Excreta

Urine (PMID: 12661026)
Feces (PMID: 27609529)

Cellular substructure

Cytoplasm (HMDB: HMDB0000128)
Membrane (HMDB: HMDB0000128)

Source

Exogenous

Exogenous (HMDB: HMDB0000128)

Endogenous

Endogenous (HMDB: HMDB0000128)

Process

Naturally occurring process

Biological process

Biochemical pathway

Metabolic pathway

Arginine and Proline Metabolism (HMDB: HMDB0000128)
Glycine and Serine Metabolism (HMDB: HMDB0000128)

Disease pathway

Argininemia (HMDB: HMDB0000128)
Creatine Deficiency, Guanidinoacetate Methyltransferase Deficiency (HMDB: HMDB0000128)
Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency) (HMDB: HMDB0000128)
S-Adenosylhomocysteine (SAH) Hydrolase Deficiency (HMDB: HMDB0000128)
Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency) (PathBank: SMP0120454)
Sarcosinemia (PathBank: SMP0120521)
Dihydropyrimidine Dehydrogenase Deficiency (DHPD) (PathBank: SMP0120694)
Hyperprolinemia Type I (PathBank: SMP0120719)
Hyperornithinemia with Gyrate Atrophy (HOGA) (PathBank: SMP0120789)
Hyperprolinemia Type II (PathBank: SMP0120501)
Non-Ketotic Hyperglycinemia (PathBank: SMP0120534)
Prolinemia Type II (PathBank: SMP0120539)
Prolidase Deficiency (PD) (PathBank: SMP0120541)
L-Arginine:Glycine Amidinotransferase Deficiency (PathBank: SMP0120572)
Hyperornithinemia-Hyperammonemia-Homocitrullinuria [HHH-syndrome] (PathBank: SMP0120790)
3-Phosphoglycerate Dehydrogenase Deficiency (PathBank: SMP0120872)
Ornithine Aminotransferase Deficiency (OAT Deficiency) (PathBank: SMP0120538)
Dimethylglycine Dehydrogenase Deficiency (PathBank: SMP0120695)
Hyperglycinemia, Non-Ketotic (PathBank: SMP0120770)
Hyperornithinemia-Hyperammonemia-Homocitrullinuria [HHH-syndrome] (PathBank: SMP0000506)

Role

Biological role

Human metabolite (HMDB: HMDB0000128)
Metabolite (HMDB: HMDB0000128)

Industrial application

Pharmaceutical industry

Biomarker

Chronic kidney disease (MarkerDB: MDB00013419)
Uremia (MarkerDB: MDB00013419)
Chronic Renal Failure (MarkerDB: MDB00013419)

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	Not Available	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	3.6 mg/mL at 15 °C	Not Available
LogP	Not Available	Not Available

Experimental Chromatographic Properties

Predicted Molecular Properties

Not Available

Predicted Chromatographic Properties

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
Guanidoacetic acid	NC(=N)NCC(O)=O	2484.0	Standard polar	33892256
Guanidoacetic acid	NC(=N)NCC(O)=O	1303.2	Standard non polar	33892256
Guanidoacetic acid	NC(=N)NCC(O)=O	1854.2	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
Guanidoacetic acid,1TMS,isomer #1	C[Si](C)(C)OC(=O)CNC(=N)N	1584.4	Semi standard non polar	33892256
Guanidoacetic acid,1TMS,isomer #2	C[Si](C)(C)NC(=N)NCC(=O)O	1724.8	Semi standard non polar	33892256
Guanidoacetic acid,1TMS,isomer #3	C[Si](C)(C)N=C(N)NCC(=O)O	1614.7	Semi standard non polar	33892256
Guanidoacetic acid,1TMS,isomer #4	C[Si](C)(C)N(CC(=O)O)C(=N)N	1649.4	Semi standard non polar	33892256
Guanidoacetic acid,2TMS,isomer #1	C[Si](C)(C)NC(=N)NCC(=O)O[Si](C)(C)C	1743.8	Semi standard non polar	33892256
Guanidoacetic acid,2TMS,isomer #1	C[Si](C)(C)NC(=N)NCC(=O)O[Si](C)(C)C	1486.2	Standard non polar	33892256
Guanidoacetic acid,2TMS,isomer #1	C[Si](C)(C)NC(=N)NCC(=O)O[Si](C)(C)C	2879.8	Standard polar	33892256
Guanidoacetic acid,2TMS,isomer #2	C[Si](C)(C)OC(=O)CN(C(=N)N)[Si](C)(C)C	1642.1	Semi standard non polar	33892256
Guanidoacetic acid,2TMS,isomer #2	C[Si](C)(C)OC(=O)CN(C(=N)N)[Si](C)(C)C	1469.1	Standard non polar	33892256
Guanidoacetic acid,2TMS,isomer #2	C[Si](C)(C)OC(=O)CN(C(=N)N)[Si](C)(C)C	2770.8	Standard polar	33892256
Guanidoacetic acid,2TMS,isomer #3	C[Si](C)(C)N=C(N)NCC(=O)O[Si](C)(C)C	1644.8	Semi standard non polar	33892256
Guanidoacetic acid,2TMS,isomer #3	C[Si](C)(C)N=C(N)NCC(=O)O[Si](C)(C)C	1411.1	Standard non polar	33892256
Guanidoacetic acid,2TMS,isomer #3	C[Si](C)(C)N=C(N)NCC(=O)O[Si](C)(C)C	2748.0	Standard polar	33892256
Guanidoacetic acid,2TMS,isomer #4	C[Si](C)(C)N(C(=N)NCC(=O)O)[Si](C)(C)C	1833.0	Semi standard non polar	33892256
Guanidoacetic acid,2TMS,isomer #4	C[Si](C)(C)N(C(=N)NCC(=O)O)[Si](C)(C)C	1541.6	Standard non polar	33892256
Guanidoacetic acid,2TMS,isomer #4	C[Si](C)(C)N(C(=N)NCC(=O)O)[Si](C)(C)C	2821.7	Standard polar	33892256
Guanidoacetic acid,2TMS,isomer #5	C[Si](C)(C)N=C(NCC(=O)O)N[Si](C)(C)C	1705.2	Semi standard non polar	33892256
Guanidoacetic acid,2TMS,isomer #5	C[Si](C)(C)N=C(NCC(=O)O)N[Si](C)(C)C	1446.6	Standard non polar	33892256
Guanidoacetic acid,2TMS,isomer #5	C[Si](C)(C)N=C(NCC(=O)O)N[Si](C)(C)C	2821.7	Standard polar	33892256
Guanidoacetic acid,2TMS,isomer #6	C[Si](C)(C)NC(=N)N(CC(=O)O)[Si](C)(C)C	1748.5	Semi standard non polar	33892256
Guanidoacetic acid,2TMS,isomer #6	C[Si](C)(C)NC(=N)N(CC(=O)O)[Si](C)(C)C	1520.6	Standard non polar	33892256
Guanidoacetic acid,2TMS,isomer #6	C[Si](C)(C)NC(=N)N(CC(=O)O)[Si](C)(C)C	2683.8	Standard polar	33892256
Guanidoacetic acid,2TMS,isomer #7	C[Si](C)(C)N=C(N)N(CC(=O)O)[Si](C)(C)C	1674.1	Semi standard non polar	33892256
Guanidoacetic acid,2TMS,isomer #7	C[Si](C)(C)N=C(N)N(CC(=O)O)[Si](C)(C)C	1470.1	Standard non polar	33892256
Guanidoacetic acid,2TMS,isomer #7	C[Si](C)(C)N=C(N)N(CC(=O)O)[Si](C)(C)C	2687.2	Standard polar	33892256
Guanidoacetic acid,3TMS,isomer #1	C[Si](C)(C)OC(=O)CNC(=N)N([Si](C)(C)C)[Si](C)(C)C	1812.1	Semi standard non polar	33892256
Guanidoacetic acid,3TMS,isomer #1	C[Si](C)(C)OC(=O)CNC(=N)N([Si](C)(C)C)[Si](C)(C)C	1610.7	Standard non polar	33892256
Guanidoacetic acid,3TMS,isomer #1	C[Si](C)(C)OC(=O)CNC(=N)N([Si](C)(C)C)[Si](C)(C)C	2398.4	Standard polar	33892256
Guanidoacetic acid,3TMS,isomer #2	C[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C)N[Si](C)(C)C	1728.2	Semi standard non polar	33892256
Guanidoacetic acid,3TMS,isomer #2	C[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C)N[Si](C)(C)C	1404.3	Standard non polar	33892256
Guanidoacetic acid,3TMS,isomer #2	C[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C)N[Si](C)(C)C	2554.3	Standard polar	33892256
Guanidoacetic acid,3TMS,isomer #3	C[Si](C)(C)NC(=N)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	1712.3	Semi standard non polar	33892256
Guanidoacetic acid,3TMS,isomer #3	C[Si](C)(C)NC(=N)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	1591.7	Standard non polar	33892256
Guanidoacetic acid,3TMS,isomer #3	C[Si](C)(C)NC(=N)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	2431.2	Standard polar	33892256
Guanidoacetic acid,3TMS,isomer #4	C[Si](C)(C)N=C(N)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	1641.8	Semi standard non polar	33892256
Guanidoacetic acid,3TMS,isomer #4	C[Si](C)(C)N=C(N)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	1458.6	Standard non polar	33892256
Guanidoacetic acid,3TMS,isomer #4	C[Si](C)(C)N=C(N)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	2599.0	Standard polar	33892256
Guanidoacetic acid,3TMS,isomer #5	C[Si](C)(C)N=C(NCC(=O)O)N([Si](C)(C)C)[Si](C)(C)C	1776.6	Semi standard non polar	33892256
Guanidoacetic acid,3TMS,isomer #5	C[Si](C)(C)N=C(NCC(=O)O)N([Si](C)(C)C)[Si](C)(C)C	1575.1	Standard non polar	33892256
Guanidoacetic acid,3TMS,isomer #5	C[Si](C)(C)N=C(NCC(=O)O)N([Si](C)(C)C)[Si](C)(C)C	2452.9	Standard polar	33892256
Guanidoacetic acid,3TMS,isomer #6	C[Si](C)(C)N(CC(=O)O)C(=N)N([Si](C)(C)C)[Si](C)(C)C	1780.5	Semi standard non polar	33892256
Guanidoacetic acid,3TMS,isomer #6	C[Si](C)(C)N(CC(=O)O)C(=N)N([Si](C)(C)C)[Si](C)(C)C	1668.6	Standard non polar	33892256
Guanidoacetic acid,3TMS,isomer #6	C[Si](C)(C)N(CC(=O)O)C(=N)N([Si](C)(C)C)[Si](C)(C)C	2365.2	Standard polar	33892256
Guanidoacetic acid,3TMS,isomer #7	C[Si](C)(C)N=C(N[Si](C)(C)C)N(CC(=O)O)[Si](C)(C)C	1695.4	Semi standard non polar	33892256
Guanidoacetic acid,3TMS,isomer #7	C[Si](C)(C)N=C(N[Si](C)(C)C)N(CC(=O)O)[Si](C)(C)C	1533.2	Standard non polar	33892256
Guanidoacetic acid,3TMS,isomer #7	C[Si](C)(C)N=C(N[Si](C)(C)C)N(CC(=O)O)[Si](C)(C)C	2373.1	Standard polar	33892256
Guanidoacetic acid,4TMS,isomer #1	C[Si](C)(C)OC(=O)CN(C(=N)N([Si](C)(C)C)[Si](C)(C)C)[Si](C)(C)C	1757.8	Semi standard non polar	33892256
Guanidoacetic acid,4TMS,isomer #1	C[Si](C)(C)OC(=O)CN(C(=N)N([Si](C)(C)C)[Si](C)(C)C)[Si](C)(C)C	1737.2	Standard non polar	33892256
Guanidoacetic acid,4TMS,isomer #1	C[Si](C)(C)OC(=O)CN(C(=N)N([Si](C)(C)C)[Si](C)(C)C)[Si](C)(C)C	2168.4	Standard polar	33892256
Guanidoacetic acid,4TMS,isomer #2	C[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C	1765.0	Semi standard non polar	33892256
Guanidoacetic acid,4TMS,isomer #2	C[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C	1561.2	Standard non polar	33892256
Guanidoacetic acid,4TMS,isomer #2	C[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C	2212.0	Standard polar	33892256
Guanidoacetic acid,4TMS,isomer #3	C[Si](C)(C)N=C(N[Si](C)(C)C)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	1715.8	Semi standard non polar	33892256
Guanidoacetic acid,4TMS,isomer #3	C[Si](C)(C)N=C(N[Si](C)(C)C)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	1525.0	Standard non polar	33892256
Guanidoacetic acid,4TMS,isomer #3	C[Si](C)(C)N=C(N[Si](C)(C)C)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	2180.4	Standard polar	33892256
Guanidoacetic acid,4TMS,isomer #4	C[Si](C)(C)N=C(N(CC(=O)O)[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C	1792.2	Semi standard non polar	33892256
Guanidoacetic acid,4TMS,isomer #4	C[Si](C)(C)N=C(N(CC(=O)O)[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C	1712.6	Standard non polar	33892256
Guanidoacetic acid,4TMS,isomer #4	C[Si](C)(C)N=C(N(CC(=O)O)[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C	2011.1	Standard polar	33892256
Guanidoacetic acid,5TMS,isomer #1	C[Si](C)(C)N=C(N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C	1809.1	Semi standard non polar	33892256
Guanidoacetic acid,5TMS,isomer #1	C[Si](C)(C)N=C(N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C	1724.7	Standard non polar	33892256
Guanidoacetic acid,5TMS,isomer #1	C[Si](C)(C)N=C(N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C	1848.3	Standard polar	33892256
Guanidoacetic acid,1TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)CNC(=N)N	1835.5	Semi standard non polar	33892256
Guanidoacetic acid,1TBDMS,isomer #2	CC(C)(C)[Si](C)(C)NC(=N)NCC(=O)O	1980.4	Semi standard non polar	33892256
Guanidoacetic acid,1TBDMS,isomer #3	CC(C)(C)[Si](C)(C)N=C(N)NCC(=O)O	1859.7	Semi standard non polar	33892256
Guanidoacetic acid,1TBDMS,isomer #4	CC(C)(C)[Si](C)(C)N(CC(=O)O)C(=N)N	1875.6	Semi standard non polar	33892256
Guanidoacetic acid,2TBDMS,isomer #1	CC(C)(C)[Si](C)(C)NC(=N)NCC(=O)O[Si](C)(C)C(C)(C)C	2218.2	Semi standard non polar	33892256
Guanidoacetic acid,2TBDMS,isomer #1	CC(C)(C)[Si](C)(C)NC(=N)NCC(=O)O[Si](C)(C)C(C)(C)C	1846.5	Standard non polar	33892256
Guanidoacetic acid,2TBDMS,isomer #1	CC(C)(C)[Si](C)(C)NC(=N)NCC(=O)O[Si](C)(C)C(C)(C)C	2749.2	Standard polar	33892256
Guanidoacetic acid,2TBDMS,isomer #2	CC(C)(C)[Si](C)(C)OC(=O)CN(C(=N)N)[Si](C)(C)C(C)(C)C	2079.4	Semi standard non polar	33892256
Guanidoacetic acid,2TBDMS,isomer #2	CC(C)(C)[Si](C)(C)OC(=O)CN(C(=N)N)[Si](C)(C)C(C)(C)C	1865.8	Standard non polar	33892256
Guanidoacetic acid,2TBDMS,isomer #2	CC(C)(C)[Si](C)(C)OC(=O)CN(C(=N)N)[Si](C)(C)C(C)(C)C	2822.9	Standard polar	33892256
Guanidoacetic acid,2TBDMS,isomer #3	CC(C)(C)[Si](C)(C)N=C(N)NCC(=O)O[Si](C)(C)C(C)(C)C	2073.2	Semi standard non polar	33892256
Guanidoacetic acid,2TBDMS,isomer #3	CC(C)(C)[Si](C)(C)N=C(N)NCC(=O)O[Si](C)(C)C(C)(C)C	1754.0	Standard non polar	33892256
Guanidoacetic acid,2TBDMS,isomer #3	CC(C)(C)[Si](C)(C)N=C(N)NCC(=O)O[Si](C)(C)C(C)(C)C	2824.4	Standard polar	33892256
Guanidoacetic acid,2TBDMS,isomer #4	CC(C)(C)[Si](C)(C)N(C(=N)NCC(=O)O)[Si](C)(C)C(C)(C)C	2234.0	Semi standard non polar	33892256
Guanidoacetic acid,2TBDMS,isomer #4	CC(C)(C)[Si](C)(C)N(C(=N)NCC(=O)O)[Si](C)(C)C(C)(C)C	1930.3	Standard non polar	33892256
Guanidoacetic acid,2TBDMS,isomer #4	CC(C)(C)[Si](C)(C)N(C(=N)NCC(=O)O)[Si](C)(C)C(C)(C)C	2636.5	Standard polar	33892256
Guanidoacetic acid,2TBDMS,isomer #5	CC(C)(C)[Si](C)(C)N=C(NCC(=O)O)N[Si](C)(C)C(C)(C)C	2209.7	Semi standard non polar	33892256
Guanidoacetic acid,2TBDMS,isomer #5	CC(C)(C)[Si](C)(C)N=C(NCC(=O)O)N[Si](C)(C)C(C)(C)C	1749.5	Standard non polar	33892256
Guanidoacetic acid,2TBDMS,isomer #5	CC(C)(C)[Si](C)(C)N=C(NCC(=O)O)N[Si](C)(C)C(C)(C)C	2631.2	Standard polar	33892256
Guanidoacetic acid,2TBDMS,isomer #6	CC(C)(C)[Si](C)(C)NC(=N)N(CC(=O)O)[Si](C)(C)C(C)(C)C	2204.0	Semi standard non polar	33892256
Guanidoacetic acid,2TBDMS,isomer #6	CC(C)(C)[Si](C)(C)NC(=N)N(CC(=O)O)[Si](C)(C)C(C)(C)C	1922.2	Standard non polar	33892256
Guanidoacetic acid,2TBDMS,isomer #6	CC(C)(C)[Si](C)(C)NC(=N)N(CC(=O)O)[Si](C)(C)C(C)(C)C	2599.3	Standard polar	33892256
Guanidoacetic acid,2TBDMS,isomer #7	CC(C)(C)[Si](C)(C)N=C(N)N(CC(=O)O)[Si](C)(C)C(C)(C)C	2072.4	Semi standard non polar	33892256
Guanidoacetic acid,2TBDMS,isomer #7	CC(C)(C)[Si](C)(C)N=C(N)N(CC(=O)O)[Si](C)(C)C(C)(C)C	1795.5	Standard non polar	33892256
Guanidoacetic acid,2TBDMS,isomer #7	CC(C)(C)[Si](C)(C)N=C(N)N(CC(=O)O)[Si](C)(C)C(C)(C)C	2741.3	Standard polar	33892256
Guanidoacetic acid,3TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)CNC(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2399.0	Semi standard non polar	33892256
Guanidoacetic acid,3TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)CNC(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2197.7	Standard non polar	33892256
Guanidoacetic acid,3TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)CNC(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2533.7	Standard polar	33892256
Guanidoacetic acid,3TBDMS,isomer #2	CC(C)(C)[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C(C)(C)C)N[Si](C)(C)C(C)(C)C	2335.8	Semi standard non polar	33892256
Guanidoacetic acid,3TBDMS,isomer #2	CC(C)(C)[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C(C)(C)C)N[Si](C)(C)C(C)(C)C	1919.2	Standard non polar	33892256
Guanidoacetic acid,3TBDMS,isomer #2	CC(C)(C)[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C(C)(C)C)N[Si](C)(C)C(C)(C)C	2562.8	Standard polar	33892256
Guanidoacetic acid,3TBDMS,isomer #3	CC(C)(C)[Si](C)(C)NC(=N)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2375.4	Semi standard non polar	33892256
Guanidoacetic acid,3TBDMS,isomer #3	CC(C)(C)[Si](C)(C)NC(=N)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2166.6	Standard non polar	33892256
Guanidoacetic acid,3TBDMS,isomer #3	CC(C)(C)[Si](C)(C)NC(=N)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2556.0	Standard polar	33892256
Guanidoacetic acid,3TBDMS,isomer #4	CC(C)(C)[Si](C)(C)N=C(N)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2259.8	Semi standard non polar	33892256
Guanidoacetic acid,3TBDMS,isomer #4	CC(C)(C)[Si](C)(C)N=C(N)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2002.5	Standard non polar	33892256
Guanidoacetic acid,3TBDMS,isomer #4	CC(C)(C)[Si](C)(C)N=C(N)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2786.2	Standard polar	33892256
Guanidoacetic acid,3TBDMS,isomer #5	CC(C)(C)[Si](C)(C)N=C(NCC(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2370.1	Semi standard non polar	33892256
Guanidoacetic acid,3TBDMS,isomer #5	CC(C)(C)[Si](C)(C)N=C(NCC(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2083.1	Standard non polar	33892256
Guanidoacetic acid,3TBDMS,isomer #5	CC(C)(C)[Si](C)(C)N=C(NCC(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2482.3	Standard polar	33892256
Guanidoacetic acid,3TBDMS,isomer #6	CC(C)(C)[Si](C)(C)N(CC(=O)O)C(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2384.2	Semi standard non polar	33892256
Guanidoacetic acid,3TBDMS,isomer #6	CC(C)(C)[Si](C)(C)N(CC(=O)O)C(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2272.8	Standard non polar	33892256
Guanidoacetic acid,3TBDMS,isomer #6	CC(C)(C)[Si](C)(C)N(CC(=O)O)C(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2497.0	Standard polar	33892256
Guanidoacetic acid,3TBDMS,isomer #7	CC(C)(C)[Si](C)(C)N=C(N[Si](C)(C)C(C)(C)C)N(CC(=O)O)[Si](C)(C)C(C)(C)C	2322.9	Semi standard non polar	33892256
Guanidoacetic acid,3TBDMS,isomer #7	CC(C)(C)[Si](C)(C)N=C(N[Si](C)(C)C(C)(C)C)N(CC(=O)O)[Si](C)(C)C(C)(C)C	2002.9	Standard non polar	33892256
Guanidoacetic acid,3TBDMS,isomer #7	CC(C)(C)[Si](C)(C)N=C(N[Si](C)(C)C(C)(C)C)N(CC(=O)O)[Si](C)(C)C(C)(C)C	2457.3	Standard polar	33892256
Guanidoacetic acid,4TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)CN(C(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2590.4	Semi standard non polar	33892256
Guanidoacetic acid,4TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)CN(C(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2485.2	Standard non polar	33892256
Guanidoacetic acid,4TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)CN(C(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2487.6	Standard polar	33892256
Guanidoacetic acid,4TBDMS,isomer #2	CC(C)(C)[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2564.0	Semi standard non polar	33892256
Guanidoacetic acid,4TBDMS,isomer #2	CC(C)(C)[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2264.6	Standard non polar	33892256
Guanidoacetic acid,4TBDMS,isomer #2	CC(C)(C)[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2466.5	Standard polar	33892256
Guanidoacetic acid,4TBDMS,isomer #3	CC(C)(C)[Si](C)(C)N=C(N[Si](C)(C)C(C)(C)C)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2499.5	Semi standard non polar	33892256
Guanidoacetic acid,4TBDMS,isomer #3	CC(C)(C)[Si](C)(C)N=C(N[Si](C)(C)C(C)(C)C)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2204.3	Standard non polar	33892256
Guanidoacetic acid,4TBDMS,isomer #3	CC(C)(C)[Si](C)(C)N=C(N[Si](C)(C)C(C)(C)C)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2461.6	Standard polar	33892256
Guanidoacetic acid,4TBDMS,isomer #4	CC(C)(C)[Si](C)(C)N=C(N(CC(=O)O)[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2574.1	Semi standard non polar	33892256
Guanidoacetic acid,4TBDMS,isomer #4	CC(C)(C)[Si](C)(C)N=C(N(CC(=O)O)[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2383.4	Standard non polar	33892256
Guanidoacetic acid,4TBDMS,isomer #4	CC(C)(C)[Si](C)(C)N=C(N(CC(=O)O)[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2354.5	Standard polar	33892256
Guanidoacetic acid,5TBDMS,isomer #1	CC(C)(C)[Si](C)(C)N=C(N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2797.0	Semi standard non polar	33892256
Guanidoacetic acid,5TBDMS,isomer #1	CC(C)(C)[Si](C)(C)N=C(N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2593.3	Standard non polar	33892256
Guanidoacetic acid,5TBDMS,isomer #1	CC(C)(C)[Si](C)(C)N=C(N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2350.8	Standard polar	33892256

Spectra

Biological Properties

Cellular Locations

Cytoplasm (predicted from logP)

Biospecimen Locations

Blood
Cellular Cytoplasm
Cerebrospinal Fluid (CSF)
Feces
Urine

Tissue Locations

Brain
Kidney
Liver
Placenta

Pathways

Name	SMPDB/PathBank	KEGG
Arginine and proline metabolism	Not Available
Glycine, serine and threonine metabolism	Not Available
Prolidase Deficiency (PD)		Not Available
Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency)		Not Available
Hyperprolinemia Type II		Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	2.8 +/- 0.9 uM	Adult (>18 years old)	Both	Normal	7296840	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Blood	Detected and Quantified	1.0-3.5 uM	Children (1-13 years old)	Not Specified	Normal	23770102	details
Blood	Detected and Quantified	0.35-8.0 uM	Children (1-13 years old)	Not Specified	Normal	23770102	details
Blood	Detected and Quantified	0.7-1.4 uM	Children (1-13 years old)	Both	Normal	11326334	details
Blood	Detected and Quantified	0.7-3.5 uM	Adult (>18 years old)	Both	Normal	11326334	details
Blood	Detected and Quantified	1.3-3.3 uM	Adult (>18 years old)	Female	Normal	15651030	details
Blood	Detected and Quantified	1.90 +/- 0.68 uM	Adult (>18 years old)	Both	Normal	22626821	details
Blood	Detected and Quantified	3.08 (0.87-3.64) uM	Adult (>18 years old)	Both	Normal	16736098	details
Blood	Detected and Quantified	19.0 (3.0-37.0) uM	Adult (>18 years old)	Not Specified	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	1.900 +/- 0.680 uM	Adult (>18 years old)	Both	Normal	12675874	details
Blood	Detected and Quantified	2.0 (0.0 - 5.0) uM	Adult (>18 years old)	Both	Normal	12661026	details
Blood	Detected and Quantified	16.8 (0.81-32.9) uM	Adult (>18 years old)	Both	Normal	18818040	details
Blood	Detected and Quantified	1.22 +/- 0.40 uM	Adult (>18 years old)	Both	Normal	3433275	details
Cellular Cytoplasm	Detected and Quantified	2.3 +/- 0.9 uM	Adult (>18 years old)	Not Specified	Normal	Geigy Scientific ...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.068-0.114 uM	Adult (>18 years old)	Female	Normal	15651030	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.05 +/- 0.03 uM	Adult (>18 years old)	Both	Normal	3433275	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Normal	27609529	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	29808030	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27543620	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21389975	details
Urine	Detected and Quantified	127.26 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	24909877	details
Urine	Detected and Quantified	35-259 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	24909877	details
Urine	Detected and Quantified	16-228 umol/mmol creatinine	Children (1 - 18 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	12.81-38.44 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	David F. Putnam C...	details
Urine	Detected and Quantified	7.69-21.36 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	David F. Putnam C...	details
Urine	Detected and Quantified	15.4 +/- 10.0 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	41.8 (10.6-97.3) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	9.33 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	Shaykhutdinov RA,...	details
Urine	Detected and Quantified	89.0 (11.0-124.0) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	12661026	details
Urine	Detected and Quantified	88.5 (18.0-159.0) umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	15369749	details
Urine	Detected and Quantified	74.0 (18.0-130.0) umol/mmol creatinine	Children (1-13 years old)	Both	Normal	15369749	details
Urine	Detected and Quantified	11.30 (7.28-20.30) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Analysis of 40 NI...	details
Urine	Detected and Quantified	13.3 (5.2-21.5) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	18818040	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	22024767	details
Urine	Detected and Quantified	4.0-220 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	23770102	details
Urine	Detected and Quantified	3-78 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	23770102	details
Urine	Detected and Quantified	10-99 umol/mmol creatinine	Children (1-13 years old)	Both	Normal	11326334	details
Urine	Detected and Quantified	<150 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	15651030	details
Urine	Detected and Quantified	93-190 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	31506554	details
Urine	Detected and Quantified	115.283 +/- 130.147 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	2.4 +/- 0.7 uM	Adult (>18 years old)	Both	Chronic renal failure	7296840	details
Blood	Detected and Quantified	1.88 +/- 0.30 uM	Adult (>18 years old)	Both	uremia	22626821	details
Blood	Detected and Quantified	17.3-27.0 uM	Adult (>18 years old)	Female	Cerebral creatine deficiency syndrome 2	15651030	details
Blood	Detected and Quantified	0.2-0.3 uM	Children (1-13 years old)	Female	Cerebral creatine deficiency syndrome 3	23770102	details
Blood	Detected and Quantified	1.1 uM	Children (1-13 years old)	Male	Cerebral creatine deficiency syndrome 1	11326334	details
Blood	Detected and Quantified	3.280 +/- 1.230 uM	Adult (>18 years old)	Both	Uremia	12675874	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	11.0-12.4 uM	Adult (>18 years old)	Female	Cerebral creatine deficiency syndrome 2	15651030	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Crohns disease	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Ulcerative colitis	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Unclassified IBD	27609529	details
Urine	Detected and Quantified	614-1624 umol/mmol creatinine	Adult (>18 years old)	Female	Cerebral creatine deficiency syndrome 2	15651030	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Schizophrenia	22024767	details
Urine	Detected and Quantified	0.9-2.6 umol/mmol creatinine	Children (1-13 years old)	Female	Cerebral creatine deficiency syndrome 3	23770102	details
Urine	Detected and Quantified	74 umol/mmol creatinine	Children (1-13 years old)	Male	Cerebral creatine deficiency syndrome 1	11326334	details
Urine	Detected and Quantified	55.592 +/- 42.373 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details

Associated Disorders and Diseases

Disease References

Chronic renal failure
Kikuchi T, Orita Y, Ando A, Mikami H, Fujii M, Okada A, Abe H: Liquid-chromatographic determination of guanidino compounds in plasma and erythrocyte of normal persons and uremic patients. Clin Chem. 1981 Nov;27(11):1899-902. [PubMed:7296840 ]
Uremia
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 ] Vanholder R, De Smet R, Glorieux G, Argiles A, Baurmeister U, Brunet P, Clark W, Cohen G, De Deyn PP, Deppisch R, Descamps-Latscha B, Henle T, Jorres A, Lemke HD, Massy ZA, Passlick-Deetjen J, Rodriguez M, Stegmayr B, Stenvinkel P, Tetta C, Wanner C, Zidek W: Review on uremic toxins: classification, concentration, and interindividual variability. Kidney Int. 2003 May;63(5):1934-43. doi: 10.1046/j.1523-1755.2003.00924.x. [PubMed:12675874 ]
Cerebral creatine deficiency syndrome 2
Caldeira Araujo H, Smit W, Verhoeven NM, Salomons GS, Silva S, Vasconcelos R, Tomas H, Tavares de Almeida I, Jakobs C, Duran M: Guanidinoacetate methyltransferase deficiency identified in adults and a child with mental retardation. Am J Med Genet A. 2005 Mar 1;133A(2):122-7. [PubMed:15651030 ]
Cerebral creatine deficiency syndrome 3
Nouioua S, Cheillan D, Zaouidi S, Salomons GS, Amedjout N, Kessaci F, Boulahdour N, Hamadouche T, Tazir M: Creatine deficiency syndrome. A treatable myopathy due to arginine-glycine amidinotransferase (AGAT) deficiency. Neuromuscul Disord. 2013 Aug;23(8):670-4. doi: 10.1016/j.nmd.2013.04.011. Epub 2013 Jun 14. [PubMed:23770102 ]
Cerebral creatine deficiency syndrome 1
Salomons GS, van Dooren SJ, Verhoeven NM, Cecil KM, Ball WS, Degrauw TJ, Jakobs C: X-linked creatine-transporter gene (SLC6A8) defect: a new creatine-deficiency syndrome. Am J Hum Genet. 2001 Jun;68(6):1497-500. doi: 10.1086/320595. Epub 2001 Apr 20. [PubMed:11326334 ]
Crohn's disease
Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Ulcerative colitis
Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Eosinophilic esophagitis
Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Schizophrenia
Yang J, Chen T, Sun L, Zhao Z, Qi X, Zhou K, Cao Y, Wang X, Qiu Y, Su M, Zhao A, Wang P, Yang P, Wu J, Feng G, He L, Jia W, Wan C: Potential metabolite markers of schizophrenia. Mol Psychiatry. 2013 Jan;18(1):67-78. doi: 10.1038/mp.2011.131. Epub 2011 Oct 25. [PubMed:22024767 ]

Associated OMIM IDs

612736 (Cerebral creatine deficiency syndrome 2)
612718 (Cerebral creatine deficiency syndrome 3)
300352 (Cerebral creatine deficiency syndrome 1)
266600 (Crohn's disease)
610247 (Eosinophilic esophagitis)
181500 (Schizophrenia)

External Links

Not Available

References

Synthesis Reference

Thalhammer, Franz; Gastner, Thomas. Preparation of creatine, creatine monohydrate and guanidinoacetic acid. Ger. Offen. (2007), 5pp.

Material Safety Data Sheet (MSDS)

Not Available

General References

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 ]
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 ]
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 ]
Schulze A: Creatine deficiency syndromes. Mol Cell Biochem. 2003 Feb;244(1-2):143-50. [PubMed:12701824 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]

Enzymes

Enzyme Details

1. Guanidinoacetate N-methyltransferase

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 + Creatine	details

Enzyme Details

2. Glycine amidinotransferase, mitochondrial

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

Reactions

L-Arginine + Glycine → Ornithine + Guanidoacetic acid	details

Showing metabocard for Guanidoacetic acid (HMDB0000128)

MOL for HMDB0000128 (Guanidoacetic acid)

3D MOL for HMDB0000128 (Guanidoacetic acid)

3D SDF for HMDB0000128 (Guanidoacetic acid)

3D-SDF for HMDB0000128 (Guanidoacetic acid)

PDB for HMDB0000128 (Guanidoacetic acid)

3D PDB for HMDB0000128 (Guanidoacetic acid)

SMILES for HMDB0000128 (Guanidoacetic acid)

INCHI for HMDB0000128 (Guanidoacetic acid)

Structure for HMDB0000128 (Guanidoacetic acid)

3D Structure for HMDB0000128 (Guanidoacetic acid)

Predicted Kovats Retention Indices

Underivatized

Derivatized

Enzymes

Reactions

Reactions