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Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References enzymes (4) Show 4 proteins XML

Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2023-02-21 17:15:03 UTC

HMDB ID

HMDB0000678

Secondary Accession Numbers

HMDB00678

Metabolite Identification

Common Name

Isovalerylglycine

Description

Isovalerylglycine (IVG) is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. In certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation. Acyl glycines are produced through the action of glycine N-acyltransferase (EC 2.3.1.13) which is an enzyme that catalyzes the chemical reaction: acyl-CoA + glycine < -- > CoA + N-acylglycine. Isovalerylglycine is a byproduct of the catabolism of the aminoacid leucine. Accumulation of isovalerylglycine occurs in Isovaleric Acidemia (IVA). IVA (OMIM/ McKusick 243500) is an autosomal recessive disorder caused by mutations in the isovaleryl-CoA dehydrogenase (EC 1.3.99.10) gene. The deficiency of this enzyme in the metabolism of leucine leads to the accumulation of a series of isovaleryl-CoA metabolites, such as isovalerylglycine. It is very important to caution for false positive results when screening for isovaleric acidemia by tandem mass spectrometry based on dried blood-spot levels of C5-acylcarnitines, including isovalerylcarnitine and its isomer, pivaloylcarnitine; pivaloylcarnitine is derived from pivalate-generating antibiotics, and has caused many false-positive results (PMID: 17850781 ). Isovalerylglycine is a biomarker for the consumption of cheese.

Structure

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


  Mrv1652307272003222D          

 24 23  0  0  0  0            999 V2000
    0.3316    1.8414    0.0000 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.0461    1.4289    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.7605    1.8414    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.7605    2.6664    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.4750    1.4289    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.1047    0.6917    0.0000 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.8453    0.6917    0.0000 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.1895    1.8414    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
    3.1895    2.6664    0.0000 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.9039    1.4289    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.9039    0.6039    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.6184    1.8414    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.9887    2.5787    0.0000 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.2482    2.5787    0.0000 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.3329    1.4289    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    5.3329    2.2539    0.0000 H   0  0  0  0  0  0  0  0  0  0  0  0
    6.0474    1.8414    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    6.2609    1.0446    0.0000 H   0  0  0  0  0  0  0  0  0  0  0  0
    6.8724    1.8414    0.0000 H   0  0  0  0  0  0  0  0  0  0  0  0
    6.2609    2.6383    0.0000 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.3329    0.6039    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.5079    0.6039    0.0000 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.3329   -0.2211    0.0000 H   0  0  0  0  0  0  0  0  0  0  0  0
    6.1579    0.6039    0.0000 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  2  3  1  0  0  0  0
  3  4  2  0  0  0  0
  3  5  1  0  0  0  0
  5  6  1  0  0  0  0
  5  7  1  0  0  0  0
  5  8  1  0  0  0  0
  8  9  1  0  0  0  0
  8 10  1  0  0  0  0
 10 11  2  0  0  0  0
 10 12  1  0  0  0  0
 12 13  1  0  0  0  0
 12 14  1  0  0  0  0
 12 15  1  0  0  0  0
 15 16  1  0  0  0  0
 15 17  1  0  0  0  0
 17 18  1  0  0  0  0
 17 19  1  0  0  0  0
 17 20  1  0  0  0  0
 15 21  1  0  0  0  0
 21 22  1  0  0  0  0
 21 23  1  0  0  0  0
 21 24  1  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0000678

> <DATABASE_NAME>
hmdb

> <SMILES>
[H]OC(=O)C([H])([H])N([H])C(=O)C([H])([H])C([H])(C([H])([H])[H])C([H])([H])[H]

> <INCHI_IDENTIFIER>
InChI=1S/C7H13NO3/c1-5(2)3-6(9)8-4-7(10)11/h5H,3-4H2,1-2H3,(H,8,9)(H,10,11)

> <INCHI_KEY>
ZRQXMKMBBMNNQC-UHFFFAOYSA-N

> <FORMULA>
C7H13NO3

> <MOLECULAR_WEIGHT>
159.183

> <EXACT_MASS>
159.089543287

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
24

> <JCHEM_AVERAGE_POLARIZABILITY>
16.39546018458692

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
2

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
2-(3-methylbutanamido)acetic acid

> <ALOGPS_LOGP>
0.35

> <JCHEM_LOGP>
0.10349465433333323

> <ALOGPS_LOGS>
-1.39

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-1

> <JCHEM_PKA>
15.905941443928317

> <JCHEM_PKA_STRONGEST_ACIDIC>
4.166378508060191

> <JCHEM_PKA_STRONGEST_BASIC>
-1.7399347523033928

> <JCHEM_POLAR_SURFACE_AREA>
66.4

> <JCHEM_REFRACTIVITY>
39.2237

> <JCHEM_ROTATABLE_BOND_COUNT>
4

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
6.50e+00 g/l

> <JCHEM_TRADITIONAL_IUPAC>
isovalerylglycine

> <JCHEM_VEBER_RULE>
0

$$$$

HEADER    PROTEIN                                 27-JUL-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   27-JUL-20         0                                  
HETATM    1  H   UNK     0       0.619   3.437   0.000  0.00  0.00           H+0
HETATM    2  O   UNK     0       1.953   2.667   0.000  0.00  0.00           O+0
HETATM    3  C   UNK     0       3.286   3.437   0.000  0.00  0.00           C+0
HETATM    4  O   UNK     0       3.286   4.977   0.000  0.00  0.00           O+0
HETATM    5  C   UNK     0       4.620   2.667   0.000  0.00  0.00           C+0
HETATM    6  H   UNK     0       3.929   1.291   0.000  0.00  0.00           H+0
HETATM    7  H   UNK     0       5.311   1.291   0.000  0.00  0.00           H+0
HETATM    8  N   UNK     0       5.954   3.437   0.000  0.00  0.00           N+0
HETATM    9  H   UNK     0       5.954   4.977   0.000  0.00  0.00           H+0
HETATM   10  C   UNK     0       7.287   2.667   0.000  0.00  0.00           C+0
HETATM   11  O   UNK     0       7.287   1.127   0.000  0.00  0.00           O+0
HETATM   12  C   UNK     0       8.621   3.437   0.000  0.00  0.00           C+0
HETATM   13  H   UNK     0       9.312   4.814   0.000  0.00  0.00           H+0
HETATM   14  H   UNK     0       7.930   4.814   0.000  0.00  0.00           H+0
HETATM   15  C   UNK     0       9.955   2.667   0.000  0.00  0.00           C+0
HETATM   16  H   UNK     0       9.955   4.207   0.000  0.00  0.00           H+0
HETATM   17  C   UNK     0      11.288   3.437   0.000  0.00  0.00           C+0
HETATM   18  H   UNK     0      11.687   1.950   0.000  0.00  0.00           H+0
HETATM   19  H   UNK     0      12.828   3.437   0.000  0.00  0.00           H+0
HETATM   20  H   UNK     0      11.687   4.925   0.000  0.00  0.00           H+0
HETATM   21  C   UNK     0       9.955   1.127   0.000  0.00  0.00           C+0
HETATM   22  H   UNK     0       8.415   1.127   0.000  0.00  0.00           H+0
HETATM   23  H   UNK     0       9.955  -0.413   0.000  0.00  0.00           H+0
HETATM   24  H   UNK     0      11.495   1.127   0.000  0.00  0.00           H+0
CONECT    1    2                                                            
CONECT    2    1    3                                                       
CONECT    3    2    4    5                                                  
CONECT    4    3                                                            
CONECT    5    3    6    7    8                                             
CONECT    6    5                                                            
CONECT    7    5                                                            
CONECT    8    5    9   10                                                  
CONECT    9    8                                                            
CONECT   10    8   11   12                                                  
CONECT   11   10                                                            
CONECT   12   10   13   14   15                                             
CONECT   13   12                                                            
CONECT   14   12                                                            
CONECT   15   12   16   17   21                                             
CONECT   16   15                                                            
CONECT   17   15   18   19   20                                             
CONECT   18   17                                                            
CONECT   19   17                                                            
CONECT   20   17                                                            
CONECT   21   15   22   23   24                                             
CONECT   22   21                                                            
CONECT   23   21                                                            
CONECT   24   21                                                            
MASTER        0    0    0    0    0    0    0    0   24    0   46    0
END

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
2-(3-Methylbutanoylamino)ethanoic acid	ChEBI
Isopentanoylglycine	ChEBI
N-Isopentanoylglycine	ChEBI
N-Isovaleroylglycine	ChEBI
2-(3-Methylbutanoylamino)ethanoate	Generator
3-Methylbutyrylglycine	HMDB
N-Isovaleryl-glycine	HMDB
[(3-Methylbutanoyl)amino]acetate	HMDB
[(3-Methylbutanoyl)amino]acetic acid	HMDB
N-Isovalerylglycine	HMDB
Isovalerylglycine	ChEBI

Chemical Formula

C₇H₁₃NO₃

Average Molecular Weight

159.183

Monoisotopic Molecular Weight

159.089543287

IUPAC Name

2-(3-methylbutanamido)acetic acid

Traditional Name

isovalerylglycine

CAS Registry Number

16284-60-9

SMILES

[H]OC(=O)C([H])([H])N([H])C(=O)C([H])([H])C([H])(C([H])([H])[H])C([H])([H])[H]

InChI Identifier

InChI=1S/C7H13NO3/c1-5(2)3-6(9)8-4-7(10)11/h5H,3-4H2,1-2H3,(H,8,9)(H,10,11)

InChI Key

ZRQXMKMBBMNNQC-UHFFFAOYSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as n-acyl-alpha amino acids. N-acyl-alpha amino acids are compounds containing an alpha amino acid which bears an acyl group at its terminal nitrogen atom.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Amino acids, peptides, and analogues

Direct Parent

N-acyl-alpha amino acids

Alternative Parents

Substituents

N-acyl-alpha-amino acid
Fatty amide
N-acyl-amine
Fatty acyl
Carboxamide group
Secondary carboxylic acid amide
Carboxylic acid
Monocarboxylic acid or derivatives
Carbonyl group
Organonitrogen compound
Organooxygen compound
Hydrocarbon derivative
Organic oxide
Organopnictogen compound
Organic oxygen compound
Organic nitrogen compound
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

N-acylglycine (CHEBI:70984 )

Ontology

Physiological effect

Health effect

Genetic disorder

Nontypified gene mutation

Organic acidemia

Glutaric aciduria type 2 (HMDB: HMDB0000678)

Nervous system disorder

Central nervous system disorder

Psychiatric disorder

Behavioral syndrome with physiological disturbances

Anorexia nervosa (HMDB: HMDB0000678)

Disposition

Biological location

Cellular substructure

Cytoplasm (HMDB: HMDB0000678)
Extracellular (HMDB: HMDB0000678)

Non-excretory biofluid

Biofluid or Excreta

Blood (HMDB: HMDB0000678)

Excreta

Biofluid or Excreta

Urine (HMDB: HMDB0000678)
Feces (PMID: 27543620)

Source

Endogenous

Endogenous (HMDB: HMDB0000678)

Greylag goose (FooDB: FOOD00368)
Guinea hen (FooDB: FOOD00373)
Ostrich (FooDB: FOOD00422)
Velvet duck (FooDB: FOOD00427)
Pheasant (FooDB: FOOD00430)
Squab (FooDB: FOOD00481)
Turkey (FooDB: FOOD00494)
Quail (FooDB: FOOD00541)
Anatidae (FooDB: FOOD00576)
Rock ptarmigan (FooDB: FOOD00597)
Columbidae (Dove, Pigeon) (FooDB: FOOD00610)
Chicken (FooDB: FOOD00329)
Mallard duck (FooDB: FOOD00353)
Emu (FooDB: FOOD00360)

Equine

Horse (FooDB: FOOD00378)

Lagomorph

Ovis

Sheep (Mutton, Lamb) (FooDB: FOOD00473)

Bovine

Venison

Deer (FooDB: FOOD00524)
Mule deer (FooDB: FOOD00348)
Elk (FooDB: FOOD00359)

Caprae

Domestic goat (FooDB: FOOD00529)

Swine

Domestic pig (FooDB: FOOD00536)
Wild boar (FooDB: FOOD00307)

Process

Naturally occurring process

Biological process

Biochemical pathway

Disease pathway

Isovaleric Aciduria (HMDB: HMDB0000678)
Ethylmalonic Encephalopathy (HMDB: HMDB0000678)
Ethylmalonic Encephalopathy (HMDB: HMDB0000678)

Role

Biological role

Metabolite (HMDB: HMDB0000678)
Metabolite (HMDB: HMDB0000678)

Industrial application

Pharmaceutical industry

Biomarker

Glutaric Acidemia Type 2 (MarkerDB: MDB00000216)
Ethylmalonic encephalopathy (MarkerDB: MDB00000216)
Isovaleric acidemia (MarkerDB: MDB00000216)
2 Methylbutyryl Co A Dehydrogenase Deficiency (MarkerDB: MDB00000216)

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	87 - 90 °C	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	Not Available	Not Available
LogP	Not Available	Not Available

Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct Type	Data Source	CCS Value (Å²)	Reference
[M-H]-	MetCCS_train_neg	138.138	30932474
[M+H]+	MetCCS_train_pos	155.136	30932474
[M-H]-	Not Available	138.138	http://allccs.zhulab.cn/database/detail?ID=AllCCS00000023

Predicted Molecular Properties

Property	Value	Source
Water Solubility	6.5 g/L	ALOGPS
logP	0.35	ALOGPS
logP	0.1	ChemAxon
logS	-1.4	ALOGPS
pKa (Strongest Acidic)	4.17	ChemAxon
pKa (Strongest Basic)	-1.7	ChemAxon
Physiological Charge	-1	ChemAxon
Hydrogen Acceptor Count	3	ChemAxon
Hydrogen Donor Count	2	ChemAxon
Polar Surface Area	66.4 Å²	ChemAxon
Rotatable Bond Count	4	ChemAxon
Refractivity	39.22 m³·mol⁻¹	ChemAxon
Polarizability	16.4 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

Predicted Chromatographic Properties

Predicted Collision Cross Sections

Predictor	Adduct Type	CCS Value (Å²)	Reference
DarkChem	[M+H]+	136.803	31661259
DarkChem	[M-H]-	133.402	31661259
AllCCS	[M+H]+	136.462	32859911
AllCCS	[M-H]-	135.416	32859911
DeepCCS	[M+H]+	138.233	30932474
DeepCCS	[M-H]-	134.746	30932474
DeepCCS	[M-2H]-	171.835	30932474
DeepCCS	[M+Na]+	147.044	30932474
AllCCS	[M+H]+	136.5	32859911
AllCCS	[M+H-H2O]+	132.6	32859911
AllCCS	[M+NH4]+	140.1	32859911
AllCCS	[M+Na]+	141.1	32859911
AllCCS	[M-H]-	135.4	32859911
AllCCS	[M+Na-2H]-	137.5	32859911
AllCCS	[M+HCOO]-	139.8	32859911

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
Isovalerylglycine	CC(C)CC(=O)NCC(O)=O	2388.8	Standard polar	33892256
Isovalerylglycine	CC(C)CC(=O)NCC(O)=O	1319.3	Standard non polar	33892256
Isovalerylglycine	CC(C)CC(=O)NCC(O)=O	1447.4	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
Isovalerylglycine,1TMS,isomer #1	CC(C)CC(=O)NCC(=O)O[Si](C)(C)C	1494.8	Semi standard non polar	33892256
Isovalerylglycine,1TMS,isomer #2	CC(C)CC(=O)N(CC(=O)O)[Si](C)(C)C	1485.1	Semi standard non polar	33892256
Isovalerylglycine,2TMS,isomer #1	CC(C)CC(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	1522.6	Semi standard non polar	33892256
Isovalerylglycine,2TMS,isomer #1	CC(C)CC(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	1526.9	Standard non polar	33892256
Isovalerylglycine,2TMS,isomer #1	CC(C)CC(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	1624.4	Standard polar	33892256
Isovalerylglycine,1TBDMS,isomer #1	CC(C)CC(=O)NCC(=O)O[Si](C)(C)C(C)(C)C	1728.4	Semi standard non polar	33892256
Isovalerylglycine,1TBDMS,isomer #2	CC(C)CC(=O)N(CC(=O)O)[Si](C)(C)C(C)(C)C	1702.9	Semi standard non polar	33892256
Isovalerylglycine,2TBDMS,isomer #1	CC(C)CC(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	1967.6	Semi standard non polar	33892256
Isovalerylglycine,2TBDMS,isomer #1	CC(C)CC(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	1922.7	Standard non polar	33892256
Isovalerylglycine,2TBDMS,isomer #1	CC(C)CC(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	1913.4	Standard polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Predicted GC-MS	Predicted GC-MS Spectrum - Isovalerylglycine GC-MS (Non-derivatized) - 70eV, Positive	splash10-0006-9200000000-30d4a8198b51e1741ecd	2017-09-01	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isovalerylglycine GC-MS (1 TMS) - 70eV, Positive	splash10-02mu-9400000000-7925a822e184ec8fe5b4	2017-10-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isovalerylglycine GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isovalerylglycine GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isovalerylglycine GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isovalerylglycine GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum

MS/MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isovalerylglycine Quattro_QQQ 10V, Positive-QTOF (Annotated)	splash10-004r-9000000000-0a3d8ca5efe599736988	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isovalerylglycine Quattro_QQQ 25V, Positive-QTOF (Annotated)	splash10-0a4i-9000000000-bdd258e50d1f36946722	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isovalerylglycine Quattro_QQQ 40V, Positive-QTOF (Annotated)	splash10-052f-9000000000-c69489d7cef77e37b417	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isovalerylglycine 20V, Negative-QTOF	splash10-00di-9000000000-f2a7577fbcf715ead09b	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isovalerylglycine 40V, Negative-QTOF	splash10-00dl-9000000000-943f0aa07bdbc49fe1ae	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isovalerylglycine 20V, Negative-QTOF	splash10-00di-9000000000-fc6f913377a676ec6911	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isovalerylglycine 10V, Negative-QTOF	splash10-00di-9300000000-a8e4825232369d335cae	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isovalerylglycine 20V, Positive-QTOF	splash10-0a7i-9600000000-f88f3b21de99e67ddf29	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isovalerylglycine 40V, Positive-QTOF	splash10-0ufr-9000000000-40ef3cce661177a7fa76	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isovalerylglycine 10V, Positive-QTOF	splash10-06ur-8900000000-a7ebb3fa9615bc15486b	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isovalerylglycine 35V, Positive-QTOF	splash10-0a4i-9000000000-1a36434c4052af0bcfe0	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isovalerylglycine 35V, Negative-QTOF	splash10-00di-9000000000-3af042b7e37d73c57609	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isovalerylglycine 10V, Negative-QTOF	splash10-00di-9100000000-a681e85779809020ebd1	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isovalerylglycine 40V, Negative-QTOF	splash10-00di-9000000000-eb0b00443a9121a49894	2021-09-20	HMDB team, MONA	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isovalerylglycine 10V, Positive-QTOF	splash10-03di-7900000000-bdfe9288fa831ea12be2	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isovalerylglycine 20V, Positive-QTOF	splash10-0a6r-9100000000-a536e4ad32d713ca358a	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isovalerylglycine 40V, Positive-QTOF	splash10-0a4i-9000000000-35336c548c7912945720	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isovalerylglycine 10V, Negative-QTOF	splash10-0a4i-1900000000-fad032ae217cd6458d13	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isovalerylglycine 20V, Negative-QTOF	splash10-0a4i-7900000000-3c2abe9b7592a2477edb	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isovalerylglycine 40V, Negative-QTOF	splash10-0ab9-9000000000-762e4bed2c894348be25	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isovalerylglycine 10V, Negative-QTOF	splash10-0ab9-9800000000-15fad814bd0853ccc685	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isovalerylglycine 20V, Negative-QTOF	splash10-00di-9000000000-e3d4167d339dc36d17cc	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isovalerylglycine 40V, Negative-QTOF	splash10-00di-9000000000-7b2fa1fa2126b1411d58	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isovalerylglycine 10V, Positive-QTOF	splash10-03dr-9400000000-11a34d1fd6d6b3c840c0	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isovalerylglycine 20V, Positive-QTOF	splash10-0a73-9000000000-0668015853aa6666c93f	2021-09-22	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Experimental 1D NMR	¹H NMR Spectrum (1D, 600 MHz, H₂O, experimental)	2012-12-04	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-16	Wishart Lab	View Spectrum
Experimental 2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, H₂O, experimental)	2012-12-05	Wishart Lab	View Spectrum

IR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-)	2023-02-03	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+)	2023-02-03	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+)	2023-02-03	FELIX lab	View Spectrum

Biological Properties

Cellular Locations

Not Available

Biospecimen Locations

Blood
Feces
Urine

Tissue Locations

Not Available

Pathways

Not Available

Name	SMPDB/PathBank	KEGG

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	0.17 +/- 0.03 uM	Newborn (0-30 days old)	Both	Normal	17850781	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27543620	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	25037050	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	29808030	details
Urine	Detected and Quantified	0.5925–7.1513 umol/mmol creatinine	Adult (25-30 years old)	Both	Not Available	23062437	details
Urine	Detected and Quantified	0.5424–5.6561 umol/mmol creatinine	Adult (25-30 years old)	Both	Not Available	23062437	details
Urine	Detected and Quantified	0.4771–4.7722 umol/mmol creatinine	Adult (25-30 years old)	Both	Not Available	23062437	details
Urine	Detected and Quantified	2-5 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	31506554	details
Urine	Detected and Quantified	3.8 +/- 0.9 umol/mmol creatinine	Adolescent (13-18 years old)	Female	Normal	10197568	details
Urine	Detected but not Quantified	Not Quantified	Adult (24-38years old)	Not Specified	Normal	20971021	details
Urine	Detected and Quantified	2.0 (0.4-4.0) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	0.41 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	21704015	details
Urine	Detected and Quantified	0.37 +/- 1.19 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	2026685	details
Urine	Detected and Quantified	2.322 +/- 1.914 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	0.213-10.157 umol/mmol creatinine	Not Specified	Not Specified	Normal	12837870	details
Urine	Detected and Quantified	<10 umol/mmol creatinine	Children (1 - 18 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	<1.15 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	28853722	details
Urine	Detected and Quantified	<14.205 umol/mmol creatinine	Children (1 - 13 years old)	Female	Normal	1246461	details
Urine	Detected and Quantified	0 - 4.5 umol/mmol creatinine	Adolescent (13-18 years old)	Both	Normal	7609451	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	40.0 (1.3-80.0) uM	Newborn (0-30 days old)	Both	Isovaleric acidemia	17850781	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	27015276	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25037050	details
Urine	Detected and Quantified	15-195 umol/mmol creatinine	Adolescent (13-18 years old)	Male	Isovaleric acidemia	Isovaleric acidem...	details
Urine	Detected and Quantified	7.7 +/- 4.8 umol/mmol creatinine	Adolescent (13-18 years old)	Female	Anorexia nervosa	10197568	details
Urine	Detected and Quantified	2.813 +/- 2.181 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details
Urine	Detected and Quantified	0.0710-1.0654 umol/mmol creatinine	Infant (0-1 year old)	Not Specified	2-Methylbutyryl-CoA dehydrogenase deficiency (SBACDD)	12837870	details
Urine	Detected and Quantified	4538.509 umol/mmol creatinine	Not Specified	Not Specified	Isovaleric acidemia	12837870	details
Urine	Detected and Quantified	0.6 umol/mmol creatinine	Adult (>18 years old)	Not Specified	Propionic acidemia	28853722	details
Urine	Detected and Quantified	4474.586 umol/mmol creatinine	Children (1 - 13 years old)	Female	Isovaleric acidemia	1246461	details
Urine	Detected and Quantified	3.4 - 36 umol/mmol creatinine	Adolescent (13-18 years old)	Both	Ethylmalonic encephalopathy	7609451	details
Urine	Detected and Quantified	511.381-795.482 umol/mmol creatinine	Newborn (0-30 days old)	Not Specified	Glutaric Aciduria II	6158623	details

Associated Disorders and Diseases

Disease References

Isovaleric acidemia
Shigematsu Y, Hata I, Tanaka Y: Stable-isotope dilution measurement of isovalerylglycine by tandem mass spectrometry in newborn screening for isovaleric acidemia. Clin Chim Acta. 2007 Nov-Dec;386(1-2):82-6. Epub 2007 Aug 19. [PubMed:17850781 ] Krieger I, Tanaka K: Therapeutic effects of glycine in isovaleric acidemia. Pediatr Res. 1976 Jan;10(1):25-9. [PubMed:1246461 ] Matern D, He M, Berry SA, Rinaldo P, Whitley CB, Madsen PP, van Calcar SC, Lussky RC, Andresen BS, Wolff JA, Vockley J: Prospective diagnosis of 2-methylbutyryl-CoA dehydrogenase deficiency in the Hmong population by newborn screening using tandem mass spectrometry. Pediatrics. 2003 Jul;112(1 Pt 1):74-8. [PubMed:12837870 ] G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium. Yasutsugu Chinen, Sadao Nakamura, Kunihito Tamashiro, Osamu Sakamoto, Kyoko Tashiro, Takahiro Inokuchi, Koichi Nakanishi (2017). Isovaleric acidemia: Therapeutic response to supplementation with glycine, /react-text l react-text: 82 -carnitine, or both in combination and a 10-year. follow-up case study. Molecular Genetics and Metabolism Reports. Volume 11, June 2017, Pages 2-5. Molecular Genetics and Metabolism Reports.
Colorectal cancer
Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ] Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Anorexia nervosa
Capo-chichi CD, Gueant JL, Lefebvre E, Bennani N, Lorentz E, Vidailhet C, Vidailhet M: Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa. Am J Clin Nutr. 1999 Apr;69(4):672-8. [PubMed:10197568 ]
Eosinophilic esophagitis
Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Propionic acidemia
Riemersma M, Hazebroek MR, Helderman-van den Enden ATJM, Salomons GS, Ferdinandusse S, Brouwers MCGJ, van der Ploeg L, Heymans S, Glatz JFC, van den Wijngaard A, Krapels IPC, Bierau J, Brunner HG: Propionic acidemia as a cause of adult-onset dilated cardiomyopathy. Eur J Hum Genet. 2017 Nov;25(11):1195-1201. doi: 10.1038/ejhg.2017.127. Epub 2017 Aug 30. [PubMed:28853722 ]
Ethylmalonic encephalopathy
Lehnert W, Ruitenbeek W: Ethylmalonic aciduria associated with progressive neurological disease and partial cytochrome c oxidase deficiency. J Inherit Metab Dis. 1993;16(3):557-9. [PubMed:7609451 ] G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Glutaric aciduria II
Gregersen N, Kolvraa S, Rasmussen K, Christensen E, Brandt NJ, Ebbesen F, Hansen FH: Biochemical studies in a patient with defects in the metabolism of acyl-CoA and sarcosine: another possible case of glutaric aciduria type II. J Inherit Metab Dis. 1980;3(3):67-72. [PubMed:6158623 ] G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Short/branched chain acyl-CoA dehydrogenase deficiency
Matern D, He M, Berry SA, Rinaldo P, Whitley CB, Madsen PP, van Calcar SC, Lussky RC, Andresen BS, Wolff JA, Vockley J: Prospective diagnosis of 2-methylbutyryl-CoA dehydrogenase deficiency in the Hmong population by newborn screening using tandem mass spectrometry. Pediatrics. 2003 Jul;112(1 Pt 1):74-8. [PubMed:12837870 ]

Associated OMIM IDs

243500 (Isovaleric acidemia)
114500 (Colorectal cancer)
606788 (Anorexia nervosa)
610247 (Eosinophilic esophagitis)
606054 (Propionic acidemia)
602473 (Ethylmalonic encephalopathy)
231680 (Glutaric aciduria II)
610006 (Short/branched chain acyl-CoA dehydrogenase deficiency)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB022177

KNApSAcK ID

Not Available

Chemspider ID

475516

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

5647

PubChem Compound

546304

PDB ID

Not Available

ChEBI ID

70984

Food Biomarker Ontology

Not Available

VMH ID

CE4968

MarkerDB ID

MDB00000216

Good Scents ID

Not Available

References

Synthesis Reference

Bondi, S.; Eissler, F. Lipoproteins and the Fatty Degeneration of Cells. Biochemische Zeitschrift (1910), 23 499-513.

Material Safety Data Sheet (MSDS)

Not Available

General References

Fries MH, Rinaldo P, Schmidt-Sommerfeld E, Jurecki E, Packman S: Isovaleric acidemia: response to a leucine load after three weeks of supplementation with glycine, L-carnitine, and combined glycine-carnitine therapy. J Pediatr. 1996 Sep;129(3):449-52. [PubMed:8804338 ]
Goodman SI, McCabe ER, Fennessey PV, Mace JW: Multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type II) with transient hypersarcosinemia and sarcosinuria; possible inherited deficiency of an electron transfer flavoprotein. Pediatr Res. 1980 Jan;14(1):12-7. [PubMed:7360517 ]
Hine DG, Hack AM, Goodman SI, Tanaka K: Stable isotope dilution analysis of isovalerylglycine in amniotic fluid and urine and its application for the prenatal diagnosis of isovaleric acidemia. Pediatr Res. 1986 Mar;20(3):222-6. [PubMed:3703611 ]
Shigematsu Y, Hata I, Tanaka Y: Stable-isotope dilution measurement of isovalerylglycine by tandem mass spectrometry in newborn screening for isovaleric acidemia. Clin Chim Acta. 2007 Nov-Dec;386(1-2):82-6. Epub 2007 Aug 19. [PubMed:17850781 ]

Enzymes

Enzyme Details

1. Glycine N-acyltransferase

General function:: Involved in glycine N-acyltransferase activity
Specific function:: Mitochondrial acyltransferase which transfers an acyl group to the N-terminus of glycine and glutamine, although much less efficiently. Can conjugate numerous substrates to form a variety of N-acylglycines, with a preference for benzoyl-CoA over phenylacetyl-CoA as acyl donors. Thereby detoxify xenobiotics, such as benzoic acid or salicylic acid, and endogenous organic acids, such as isovaleric acid.
Gene Name:: GLYAT
Uniprot ID:: Q6IB77
Molecular weight:: 18506.33

Enzyme Details

2. Glycine N-acyltransferase-like protein 1

General function:: Involved in glycine N-acyltransferase activity
Specific function:: Acyltransferase which transfers an acyl group to the N-terminus of glutamine. Can use phenylacetyl-CoA as an acyl donor.
Gene Name:: GLYATL1
Uniprot ID:: Q969I3
Molecular weight:: 35100.895

Enzyme Details

3. Glycine N-acyltransferase-like protein 2

General function:: Involved in glycine N-acyltransferase activity
Specific function:: Mitochondrial acyltransferase which transfers the acyl group to the N-terminus of glycine. Conjugates numerous substrates, such as arachidonoyl-CoA and saturated medium and long-chain acyl-CoAs ranging from chain-length C8:0-CoA to C18:0-CoA, to form a variety of N-acylglycines. Shows a preference for monounsaturated fatty acid oleoyl-CoA (C18:1-CoA) as an acyl donor. Does not exhibit any activity toward C22:6-CoA and chenodeoxycholoyl-CoA, nor toward serine or alanine.
Gene Name:: GLYATL2
Uniprot ID:: Q8WU03
Molecular weight:: 34277.055

Enzyme Details

4. Glycine N-acyltransferase-like protein 3

General function:: Involved in glycine N-acyltransferase activity
Specific function:: Acyltransferase which transfers the acyl group to the N- terminus of glycine
Gene Name:: GLYATL3
Uniprot ID:: Q5SZD4
Molecular weight:: 32703.3

Showing metabocard for Isovalerylglycine (HMDB0000678)

MOL for HMDB0000678 (Isovalerylglycine)

3D MOL for HMDB0000678 (Isovalerylglycine)

3D SDF for HMDB0000678 (Isovalerylglycine)

3D-SDF for HMDB0000678 (Isovalerylglycine)

PDB for HMDB0000678 (Isovalerylglycine)

3D PDB for HMDB0000678 (Isovalerylglycine)

SMILES for HMDB0000678 (Isovalerylglycine)

INCHI for HMDB0000678 (Isovalerylglycine)

Structure for HMDB0000678 (Isovalerylglycine)

3D Structure for HMDB0000678 (Isovalerylglycine)

Experimental Collision Cross Sections

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

Underivatized

Derivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra

IR Spectra

Enzymes