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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 17:15:07 UTC

HMDB ID

HMDB0000730

Secondary Accession Numbers

HMDB00730

Metabolite Identification

Common Name

Isobutyrylglycine

Description

Isobutyrylglycine 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. Isobutyrylglycine is identified in large amount in urine of patients with isobutyryl-CoA dehydrogenase deficiency. Isobutyryl-CoA dehydrogenase deficiency is a disorder caused by the deficiency of isobutyryl-CoA dehydrogenase that is involved in the catabolism of the branched-chain amino acid valine (PMID 15505379 ). Moreover, Isobutyrylglycine is found to be associated with ethylmalonic encephalopathy and propionic acidemia, which are also inborn errors of metabolism. Isobutyrylglycine is a biomarker for the consumption of cheese.

Structure

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

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
Isobutanoylglycine	ChEBI
N-Isobutanoylglycine	ChEBI
N-(1-Methylpropyl)glycine	HMDB
N-Isobutyrylglycine	HMDB
Isobutyrylglycine	ChEBI

Chemical Formula

C₆H₁₁NO₃

Average Molecular Weight

145.1564

Monoisotopic Molecular Weight

145.073893223

IUPAC Name

2-(2-methylpropanamido)acetic acid

Traditional Name

isobutyrylglycine

CAS Registry Number

15926-18-8

SMILES

CC(C)C(=O)NCC(O)=O

InChI Identifier

InChI=1S/C6H11NO3/c1-4(2)6(10)7-3-5(8)9/h4H,3H2,1-2H3,(H,7,10)(H,8,9)

InChI Key

DCICDMMXFIELDF-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
Secondary carboxylic acid amide
Carboxamide group
Monocarboxylic acid or derivatives
Carboxylic acid
Organic nitrogen compound
Organic oxygen compound
Organopnictogen compound
Organic oxide
Hydrocarbon derivative
Organooxygen compound
Organonitrogen compound
Carbonyl group
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

N-acylglycine (CHEBI:70979 )

Ontology

Physiological effect

Health effect

Genetic disorder

Nontypified gene mutation

Organic acidemia

Glutaric aciduria type 2 (HMDB: HMDB0000730)

Disposition

Biological location

Cellular substructure

Cytoplasm (HMDB: HMDB0000730)
Extracellular (HMDB: HMDB0000730)

Non-excretory biofluid

Biofluid or Excreta

Blood (HMDB: HMDB0000730)

Excreta

Biofluid or Excreta

Urine (HMDB: HMDB0000730)
Feces (PMID: 27543620)

Source

Endogenous

Endogenous (HMDB: HMDB0000730)

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

Ethylmalonic Encephalopathy (HMDB: HMDB0000730)
Ethylmalonic Encephalopathy (HMDB: HMDB0000730)

Role

Biological role

Metabolite (HMDB: HMDB0000730)
Metabolite (HMDB: HMDB0000730)

Industrial application

Pharmaceutical industry

Biomarker

Glutaric Acidemia Type 2 (MarkerDB: MDB00000239)
Ethylmalonic encephalopathy (MarkerDB: MDB00000239)

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	82.5 °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	132.067	30932474
[M-H]-	Not Available	132.067	http://allccs.zhulab.cn/database/detail?ID=AllCCS00000030

Predicted Molecular Properties

Property	Value	Source
Water Solubility	17.9 g/L	ALOGPS
logP	-0.11	ALOGPS
logP	-0.085	ChemAxon
logS	-0.91	ALOGPS
pKa (Strongest Acidic)	4.06	ChemAxon
pKa (Strongest Basic)	-1.8	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	3	ChemAxon
Refractivity	34.65 m³·mol⁻¹	ChemAxon
Polarizability	14.57 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]+	132.077	31661259
DarkChem	[M-H]-	127.542	31661259
AllCCS	[M+H]+	133.428	32859911
AllCCS	[M-H]-	130.878	32859911
DeepCCS	[M+H]+	130.898	30932474
DeepCCS	[M-H]-	127.363	30932474
DeepCCS	[M-2H]-	164.298	30932474
DeepCCS	[M+Na]+	139.461	30932474
AllCCS	[M+H]+	133.4	32859911
AllCCS	[M+H-H2O]+	129.5	32859911
AllCCS	[M+NH4]+	137.1	32859911
AllCCS	[M+Na]+	138.1	32859911
AllCCS	[M-H]-	130.9	32859911
AllCCS	[M+Na-2H]-	133.3	32859911
AllCCS	[M+HCOO]-	136.0	32859911

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
Isobutyrylglycine	CC(C)C(=O)NCC(O)=O	2295.0	Standard polar	33892256
Isobutyrylglycine	CC(C)C(=O)NCC(O)=O	1247.7	Standard non polar	33892256
Isobutyrylglycine	CC(C)C(=O)NCC(O)=O	1350.4	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
Isobutyrylglycine,1TMS,isomer #1	CC(C)C(=O)NCC(=O)O[Si](C)(C)C	1380.6	Semi standard non polar	33892256
Isobutyrylglycine,1TMS,isomer #2	CC(C)C(=O)N(CC(=O)O)[Si](C)(C)C	1395.2	Semi standard non polar	33892256
Isobutyrylglycine,2TMS,isomer #1	CC(C)C(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	1437.7	Semi standard non polar	33892256
Isobutyrylglycine,2TMS,isomer #1	CC(C)C(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	1410.8	Standard non polar	33892256
Isobutyrylglycine,2TMS,isomer #1	CC(C)C(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	1547.3	Standard polar	33892256
Isobutyrylglycine,1TBDMS,isomer #1	CC(C)C(=O)NCC(=O)O[Si](C)(C)C(C)(C)C	1619.5	Semi standard non polar	33892256
Isobutyrylglycine,1TBDMS,isomer #2	CC(C)C(=O)N(CC(=O)O)[Si](C)(C)C(C)(C)C	1622.7	Semi standard non polar	33892256
Isobutyrylglycine,2TBDMS,isomer #1	CC(C)C(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	1874.6	Semi standard non polar	33892256
Isobutyrylglycine,2TBDMS,isomer #1	CC(C)C(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	1833.0	Standard non polar	33892256
Isobutyrylglycine,2TBDMS,isomer #1	CC(C)C(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	1832.0	Standard polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Predicted GC-MS	Predicted GC-MS Spectrum - Isobutyrylglycine GC-MS (Non-derivatized) - 70eV, Positive	splash10-00dl-9100000000-91e12bffc069cc1fe9a0	2017-09-01	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isobutyrylglycine GC-MS (1 TMS) - 70eV, Positive	splash10-00di-9100000000-5281458abfc09bd42ce1	2017-10-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isobutyrylglycine GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isobutyrylglycine GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isobutyrylglycine GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isobutyrylglycine GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isobutyrylglycine 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 - Isobutyrylglycine Quattro_QQQ 10V, Positive-QTOF (Annotated)	splash10-00fr-9000000000-f1d95bb7599bb8f6cd73	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isobutyrylglycine Quattro_QQQ 25V, Positive-QTOF (Annotated)	splash10-0006-9000000000-b41069b8dbfe4b85b346	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isobutyrylglycine Quattro_QQQ 40V, Positive-QTOF (Annotated)	splash10-0006-9000000000-85cb61c87f73b734a9e5	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isobutyrylglycine 35V, Positive-QTOF	splash10-0006-9000000000-3fc2c9bdad8d5402bc37	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isobutyrylglycine 35V, Negative-QTOF	splash10-00di-9000000000-25d45687b4de12d7278a	2021-09-20	HMDB team, MONA	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isobutyrylglycine 10V, Positive-QTOF	splash10-006t-4900000000-56e60896238c44e6c163	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isobutyrylglycine 20V, Positive-QTOF	splash10-00dl-9300000000-b49a0fcfcc97adc64a23	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isobutyrylglycine 40V, Positive-QTOF	splash10-0596-9000000000-19f4499051d30aa160e8	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isobutyrylglycine 10V, Negative-QTOF	splash10-0006-0900000000-96c02d5f830c0ad4ba52	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isobutyrylglycine 20V, Negative-QTOF	splash10-0f6x-1900000000-be6409aa473bdd7dc417	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isobutyrylglycine 40V, Negative-QTOF	splash10-05fr-9000000000-2656458192decd1b1fbf	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isobutyrylglycine 10V, Negative-QTOF	splash10-0006-0900000000-ea3f3c5f4359d8e7d671	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isobutyrylglycine 20V, Negative-QTOF	splash10-0kfw-9300000000-2add86a545e7ecd4a0ec	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isobutyrylglycine 40V, Negative-QTOF	splash10-0596-9000000000-d0a390fd6e37ef2d8a63	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isobutyrylglycine 10V, Positive-QTOF	splash10-0006-9100000000-505e17d2533aeaf498f8	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isobutyrylglycine 20V, Positive-QTOF	splash10-0006-9000000000-28a4e51c194d7751c731	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isobutyrylglycine 40V, Positive-QTOF	splash10-0006-9000000000-dda745bb6fc8ca1dced5	2021-09-24	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Experimental 1D NMR	¹H NMR Spectrum (1D, 500 MHz, CDCl₃, experimental)	2012-12-04	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Experimental 2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, CD₃OD, 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

Cytoplasm (predicted from logP)

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 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	29808030	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27543620	details
Urine	Detected and Quantified	0-5 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	31506554	details
Urine	Detected but not Quantified	Not Quantified	Adult (24-38years old)	Not Specified	Normal	20971021	details
Urine	Detected and Quantified	0.48 (0-1.92) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	21704015	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21389975	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	<2.9 umol/mmol creatinine	Children (1 - 18 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0.2157–1.6217 umol/mmol creatinine	Adult (25-30 years old)	Both	Not Available	23062437	details
Urine	Detected and Quantified	0.1742–1.2490 umol/mmol creatinine	Adult (25-30 years old)	Both	Not Available	23062437	details
Urine	Detected and Quantified	0.1643–0.8386 umol/mmol creatinine	Adult (25-30 years old)	Both	Not Available	23062437	details
Urine	Detected and Quantified	<0.99 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	28853722	details
Urine	Detected and Quantified	<8.6 umol/mmol creatinine	Children (1 - 13 years old)	Female	Normal	9667231	details
Urine	Detected and Quantified	0.5 - 3.8 umol/mmol creatinine	Adolescent (13-18 years old)	Both	Normal	7609451	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Urine	Detected and Quantified	1.791-27.339 umol/mmol creatinine	Newborn (0-30 days old)	Both	Isobutyryl-CoA Dehydrogenase Deficiency	17304052	details
Urine	Detected and Quantified	1.558-7.477 umol/mmol creatinine	Infant (0-1 year old)	Male	Isobutyryl-CoA Dehydrogenase Deficiency	17304052	details
Urine	Detected and Quantified	0.0234-1.106 umol/mmol creatinine	Newborn (0-30 days old)	Not Specified	Short Chain Acyl-Coa Dehydrogenase Deficiency	17304052	details
Urine	Detected and Quantified	1.69 umol/mmol creatinine	Adult (>18 years old)	Not Specified	Propionic acidemia	28853722	details
Urine	Detected and Quantified	17.4 umol/mmol creatinine	Children (1 - 13 years old)	Female	Ethylmalonic encephalopathy	9667231	details
Urine	Detected and Quantified	31.155 umol/mmol creatinine	Newborn (0-30 days old)	Not Specified	Glutaric Aciduria II	6158623	details
Urine	Detected and Quantified	4.5 - 33 umol/mmol creatinine	Adolescent (13-18 years old)	Both	Ethylmalonic encephalopathy	7609451	details

Associated Disorders and Diseases

Disease References

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
Nowaczyk MJ, Lehotay DC, Platt BA, Fisher L, Tan R, Phillips H, Clarke JT: Ethylmalonic and methylsuccinic aciduria in ethylmalonic encephalopathy arise from abnormal isoleucine metabolism. Metabolism. 1998 Jul;47(7):836-9. [PubMed:9667231 ] 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.
Isobutyryl-CoA Dehydrogenase Deficiency
Oglesbee D, He M, Majumder N, Vockley J, Ahmad A, Angle B, Burton B, Charrow J, Ensenauer R, Ficicioglu CH, Keppen LD, Marsden D, Tortorelli S, Hahn SH, Matern D: Development of a newborn screening follow-up algorithm for the diagnosis of isobutyryl-CoA dehydrogenase deficiency. Genet Med. 2007 Feb;9(2):108-16. doi: 10.1097GIM.0b013e31802f78d6. [PubMed:17304052 ]
Short Chain Acyl-Coa Dehydrogenase Deficiency
Oglesbee D, He M, Majumder N, Vockley J, Ahmad A, Angle B, Burton B, Charrow J, Ensenauer R, Ficicioglu CH, Keppen LD, Marsden D, Tortorelli S, Hahn SH, Matern D: Development of a newborn screening follow-up algorithm for the diagnosis of isobutyryl-CoA dehydrogenase deficiency. Genet Med. 2007 Feb;9(2):108-16. doi: 10.1097GIM.0b013e31802f78d6. [PubMed:17304052 ]

Associated OMIM IDs

606054 (Propionic acidemia)
602473 (Ethylmalonic encephalopathy)
231680 (Glutaric aciduria II)
611283 (Isobutyryl-CoA Dehydrogenase Deficiency)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB022208

KNApSAcK ID

C00052325

Chemspider ID

9030891

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

5698

PubChem Compound

10855600

PDB ID

Not Available

ChEBI ID

70979

Food Biomarker Ontology

Not Available

VMH ID

CE4969

MarkerDB ID

MDB00000239

Good Scents ID

Not Available

References

Synthesis Reference

Katz, J.; Lieberman, I.; Barker, H. A. Formation of propionyl-, butyryl-, and other acylglycines by enzymes of Clostridium kluyveri. Journal of Biological Chemistry (1953), 200 431-41.

Material Safety Data Sheet (MSDS)

Not Available

General References

Sass JO, Sander S, Zschocke J: Isobutyryl-CoA dehydrogenase deficiency: isobutyrylglycinuria and ACAD8 gene mutations in two infants. J Inherit Metab Dis. 2004;27(6):741-5. [PubMed:15505379 ]
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 ]

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 Isobutyrylglycine (HMDB0000730)

MOL for HMDB0000730 (Isobutyrylglycine)

3D MOL for HMDB0000730 (Isobutyrylglycine)

3D SDF for HMDB0000730 (Isobutyrylglycine)

3D-SDF for HMDB0000730 (Isobutyrylglycine)

PDB for HMDB0000730 (Isobutyrylglycine)

3D PDB for HMDB0000730 (Isobutyrylglycine)

SMILES for HMDB0000730 (Isobutyrylglycine)

INCHI for HMDB0000730 (Isobutyrylglycine)

Structure for HMDB0000730 (Isobutyrylglycine)

3D Structure for HMDB0000730 (Isobutyrylglycine)

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