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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 20:55:53 UTC

HMDB ID

HMDB0000959

Secondary Accession Numbers

HMDB00959

Metabolite Identification

Common Name

Tiglylglycine

Description

Tiglylglycine 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. Tiglylglycine is an intermediate product of the catabolism of isoleucine. An elevated level of tiglylglycine is identified in urine of patients with beta-ketothiolase deficiency or with disorders of propionate metabolism (PMID 7923765 ). Tiglyglycine 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
N-Tiglylglycine	ChEBI
(e)-N-(2-Methyl-1-oxo-2-butenyl)-glycine	HMDB
N-((e)-2-Methyl-but-2-enoyl)-glycine	HMDB
N-Tigloylglycine	HMDB

Chemical Formula

C₇H₁₁NO₃

Average Molecular Weight

157.1671

Monoisotopic Molecular Weight

157.073893223

IUPAC Name

2-[(2E)-2-methylbut-2-enamido]acetic acid

Traditional Name

[(2E)-2-methylbut-2-enamido]acetic acid

CAS Registry Number

35842-45-6

SMILES

C\C=C(/C)C(=O)NCC(O)=O

InChI Identifier

InChI=1S/C7H11NO3/c1-3-5(2)7(11)8-4-6(9)10/h3H,4H2,1-2H3,(H,8,11)(H,9,10)/b5-3+

InChI Key

WRUSVQOKJIDBLP-HWKANZROSA-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
N-acyl-amine
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:73018 )

Ontology

Not Available

Urine (PMID: 8087979)

Cellular substructure

Cytoplasm (HMDB: HMDB0000959)
Extracellular (HMDB: HMDB0000959)

Source

Endogenous

Endogenous (HMDB: HMDB0000959)

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)
Greylag goose (FooDB: FOOD00368)
Guinea hen (FooDB: FOOD00373)
Ostrich (FooDB: FOOD00422)
Velvet duck (FooDB: FOOD00427)

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)

Equine

Horse (FooDB: FOOD00378)

Process

Naturally occurring process

Biological process

Biochemical pathway

Disease pathway

2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency (HMDB: HMDB0000959)
Beta-Ketothiolase Deficiency (HMDB: HMDB0000959)

Role

Biological role

Metabolite (HMDB: HMDB0000959)
Metabolite (HMDB: HMDB0000959)

Industrial application

Pharmaceutical industry

Biomarker

Ketosis (MarkerDB: MDB00000292)

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	Not Available	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]-	Not Available	142.931	http://allccs.zhulab.cn/database/detail?ID=AllCCS00001751

Predicted Molecular Properties

Property	Value	Source
Water Solubility	7.52 g/L	ALOGPS
logP	0.33	ALOGPS
logP	0.21	ChemAxon
logS	-1.3	ALOGPS
pKa (Strongest Acidic)	4.09	ChemAxon
pKa (Strongest Basic)	-0.49	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	40.13 m³·mol⁻¹	ChemAxon
Polarizability	15.97 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
AllCCS	[M+H]+	136.327	32859911
AllCCS	[M-H]-	133.475	32859911
DeepCCS	[M+H]+	134.142	30932474
DeepCCS	[M-H]-	130.314	30932474
DeepCCS	[M-2H]-	167.68	30932474
DeepCCS	[M+Na]+	143.219	30932474
AllCCS	[M+H]+	136.3	32859911
AllCCS	[M+H-H2O]+	132.4	32859911
AllCCS	[M+NH4]+	140.0	32859911
AllCCS	[M+Na]+	141.1	32859911
AllCCS	[M-H]-	133.5	32859911
AllCCS	[M+Na-2H]-	135.5	32859911
AllCCS	[M+HCOO]-	137.7	32859911

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
Tiglylglycine	C\C=C(/C)C(=O)NCC(O)=O	2488.7	Standard polar	33892256
Tiglylglycine	C\C=C(/C)C(=O)NCC(O)=O	1330.3	Standard non polar	33892256
Tiglylglycine	C\C=C(/C)C(=O)NCC(O)=O	1536.7	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
Tiglylglycine,1TMS,isomer #1	C/C=C(\C)C(=O)NCC(=O)O[Si](C)(C)C	1584.4	Semi standard non polar	33892256
Tiglylglycine,1TMS,isomer #2	C/C=C(\C)C(=O)N(CC(=O)O)[Si](C)(C)C	1563.8	Semi standard non polar	33892256
Tiglylglycine,2TMS,isomer #1	C/C=C(\C)C(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	1586.3	Semi standard non polar	33892256
Tiglylglycine,2TMS,isomer #1	C/C=C(\C)C(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	1559.6	Standard non polar	33892256
Tiglylglycine,2TMS,isomer #1	C/C=C(\C)C(=O)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C	1736.8	Standard polar	33892256
Tiglylglycine,1TBDMS,isomer #1	C/C=C(\C)C(=O)NCC(=O)O[Si](C)(C)C(C)(C)C	1812.8	Semi standard non polar	33892256
Tiglylglycine,1TBDMS,isomer #2	C/C=C(\C)C(=O)N(CC(=O)O)[Si](C)(C)C(C)(C)C	1801.4	Semi standard non polar	33892256
Tiglylglycine,2TBDMS,isomer #1	C/C=C(\C)C(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2048.5	Semi standard non polar	33892256
Tiglylglycine,2TBDMS,isomer #1	C/C=C(\C)C(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	1966.0	Standard non polar	33892256
Tiglylglycine,2TBDMS,isomer #1	C/C=C(\C)C(=O)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C	2010.0	Standard polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Predicted GC-MS	Predicted GC-MS Spectrum - Tiglylglycine GC-MS (Non-derivatized) - 70eV, Positive	splash10-001i-9200000000-092824f74c108907a49e	2017-09-01	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Tiglylglycine GC-MS (1 TMS) - 70eV, Positive	splash10-00e9-9100000000-5d3935f85eccdefefb59	2017-10-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Tiglylglycine GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Tiglylglycine GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Tiglylglycine GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Tiglylglycine GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Tiglylglycine GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-06	Wishart Lab	View Spectrum

MS/MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental LC-MS/MS	LC-MS/MS Spectrum - Tiglylglycine Quattro_QQQ 10V, Positive-QTOF (Annotated)	splash10-004i-9000000000-4f7bd3c47a6d77fcc178	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Tiglylglycine Quattro_QQQ 25V, Positive-QTOF (Annotated)	splash10-004i-9000000000-7ee9df3c83b8760c7e09	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Tiglylglycine Quattro_QQQ 40V, Positive-QTOF (Annotated)	splash10-03fr-9000000000-da47f9a5f3d8fdcaf90a	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Tiglylglycine LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positive-QTOF	splash10-001i-9000000000-6fdd0ba2be939d0a161a	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Tiglylglycine LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative-QTOF	splash10-0a4i-1900000000-2cd6e4c6ab2eeb847328	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Tiglylglycine LC-ESI-QTOF , negative-QTOF	splash10-0a4i-1900000000-2cd6e4c6ab2eeb847328	2017-09-14	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Tiglylglycine LC-ESI-QTOF , positive-QTOF	splash10-001i-9000000000-6fdd0ba2be939d0a161a	2017-09-14	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Tiglylglycine 20V, Positive-QTOF	splash10-0a4i-9000000000-6640ab0bba7f488fb1b7	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Tiglylglycine 10V, Positive-QTOF	splash10-001i-9000000000-52faded097646da69ec7	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Tiglylglycine 10V, Negative-QTOF	splash10-0bt9-2900000000-605b7b7b40a77223a8f9	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Tiglylglycine 40V, Positive-QTOF	splash10-0a4i-9000000000-86f6f96695e2d9fe9a9a	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Tiglylglycine 40V, Negative-QTOF	splash10-0006-9000000000-ddf9655db6a774124c69	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Tiglylglycine 20V, Negative-QTOF	splash10-03di-7900000000-ecab07cf409aa411046f	2021-09-20	HMDB team, MONA	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Tiglylglycine 10V, Positive-QTOF	splash10-0a59-5900000000-7e583345ae99d6656bad	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Tiglylglycine 20V, Positive-QTOF	splash10-0a59-9300000000-25bd55529a83deba5ea2	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Tiglylglycine 40V, Positive-QTOF	splash10-0a4i-9000000000-c359dba2d89f41f03224	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Tiglylglycine 10V, Negative-QTOF	splash10-0a4i-0900000000-3376764fa5c9bcdde347	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Tiglylglycine 20V, Negative-QTOF	splash10-0a4i-2900000000-4dde97028084b16db2bd	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Tiglylglycine 40V, Negative-QTOF	splash10-0ab9-9000000000-7185fafe7ab0f33f5c5d	2017-09-01	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Tiglylglycine 10V, Positive-QTOF	splash10-0a4i-5900000000-4d9d366aa5db2c493277	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Tiglylglycine 20V, Positive-QTOF	splash10-0a4i-9000000000-69e0efbc2e71d768a57c	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Tiglylglycine 40V, Positive-QTOF	splash10-0a4r-9000000000-fe61e613b1d7fd7c77e5	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Tiglylglycine 10V, Negative-QTOF	splash10-052r-0900000000-c31bc851b562c2b42930	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Tiglylglycine 20V, Negative-QTOF	splash10-05fu-9300000000-2ec83a972a0157664b35	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Tiglylglycine 40V, Negative-QTOF	splash10-0a4i-9000000000-372794b6f97a5c4c1481	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, H₂O, experimental)	2012-12-04	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, 100 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, 200 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, 300 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, 400 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, 500 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, 600 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, 700 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, 800 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, 900 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
Predicted 1D NMR	¹³C 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

Cytoplasm

Biospecimen Locations

Blood
Urine

Tissue Locations

Not Available

Pathways

Not Available

Name	SMPDB/PathBank	KEGG

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Urine	Detected and Quantified	0-3.8 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	21704015	details
Urine	Detected and Quantified	<7 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	28853722	details
Urine	Detected and Quantified	0.94-1.8 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	27012787	details
Urine	Detected and Quantified	0.79 (0.55-0.97) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Analysis of 40 NI...	details
Urine	Detected and Quantified	<15.62 umol/mmol creatinine	Children (1 - 18 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21389975	details
Urine	Detected and Quantified	0-0.1 umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	31963255	details
Urine	Detected and Quantified	<5 umol/mmol creatinine	Not Specified	Not Specified	Normal	16148061	details
Urine	Detected and Quantified	0.1 (0.1-7.6) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	8087979	details
Urine	Detected and Quantified	2.5 (0.1-6.7) umol/mmol creatinine	Children (1-13 years old)	Both	Normal	8087979	details
Urine	Detected and Quantified	2.1 (0.1-5.9) umol/mmol creatinine	Children (1-13 years old)	Both	Normal	8087979	details
Urine	Detected and Quantified	1.8 (0.1-6.3) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	8087979	details
Urine	Detected and Quantified	0.78-1.2 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	27012787	details
Urine	Detected and Quantified	0.5688–5.0342 umol/mmol creatinine	Adult (25-30 years old)	Both	Not Available	23062437	details
Urine	Detected and Quantified	0.5598–3.5193 umol/mmol creatinine	Adult (25-30 years old)	Both	Not Available	23062437	details
Urine	Detected and Quantified	0.4703–3.1901 umol/mmol creatinine	Adult (25-30 years old)	Both	Not Available	23062437	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Expected but not Quantified	Not Quantified	Not Specified	Not Specified	Cancer patients undergoing total body irradiation	31424920	details
Urine	Detected and Quantified	6.7-45.8 umol/mmol creatinine	Children (1-13 years old)	Male	Beta-ketothiolase deficiency	23430882	details
Urine	Detected and Quantified	87 umol/mmol creatinine	Newborn (0-30 days old)	Not Specified	Beta-ketothiolase deficiency	25689098	details
Urine	Detected and Quantified	930.484-993.797 umol/mmol creatinine	Children (1 - 13 years old)	Female	Ketosis	B. Middleton et a...	details
Urine	Detected and Quantified	1.0 (0.0-2.0) umol/mmol creatinine	Adult (>18 years old)	Both	Beta-ketothiolase deficiency	MetaGene: Metabol...	details
Urine	Detected and Quantified	500.0 (0.0-1000.0) umol/mmol creatinine	Children (1-13 years old)	Both	Beta-Ketothiolase deficiency	MetaGene: Metabol...	details
Urine	Detected and Quantified	6.44 umol/mmol creatinine	Adult (>18 years old)	Not Specified	Propionic acidemia	28853722	details
Urine	Detected and Quantified	21 umol/mmol creatinine	Children (1-13 years old)	Female	2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency	16148061	details
Urine	Detected and Quantified	145-440 umol/mmol creatinine	Infant (0-1 year old)	Male	2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency	16148061	details

Associated Disorders and Diseases

Disease References

Beta-ketothiolase deficiency
Fukao T, Maruyama S, Ohura T, Hasegawa Y, Toyoshima M, Haapalainen AM, Kuwada N, Imamura M, Yuasa I, Wierenga RK, Yamaguchi S, Kondo N: Three Japanese Patients with Beta-Ketothiolase Deficiency Who Share a Mutation, c.431A>C (H144P) in ACAT1 : Subtle Abnormality in Urinary Organic Acid Analysis and Blood Acylcarnitine Analysis Using Tandem Mass Spectrometry. JIMD Rep. 2012;3:107-15. doi: 10.1007/8904_2011_72. Epub 2011 Sep 6. [PubMed:23430882 ] Scolamiero E, Cozzolino C, Albano L, Ansalone A, Caterino M, Corbo G, di Girolamo MG, Di Stefano C, Durante A, Franzese G, Franzese I, Gallo G, Giliberti P, Ingenito L, Ippolito G, Malamisura B, Mazzeo P, Norma A, Ombrone D, Parenti G, Pellecchia S, Pecce R, Pierucci I, Romanelli R, Rossi A, Siano M, Stoduto T, Villani GR, Andria G, Salvatore F, Frisso G, Ruoppolo M: Targeted metabolomics in the expanded newborn screening for inborn errors of metabolism. Mol Biosyst. 2015 Jun;11(6):1525-35. doi: 10.1039/c4mb00729h. [PubMed:25689098 ] G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Ketosis
B. Middleton et al. (1986). B. Middleton et al. 3-Ketothiolase deficiency. Eur J Pediatr (1986) 144:586-589. Eur J Pediatr.
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 ]
2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency
Perez-Cerda C, Garcia-Villoria J, Ofman R, Sala PR, Merinero B, Ramos J, Garcia-Silva MT, Beseler B, Dalmau J, Wanders RJ, Ugarte M, Ribes A: 2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency: an X-linked inborn error of isoleucine metabolism that may mimic a mitochondrial disease. Pediatr Res. 2005 Sep;58(3):488-91. [PubMed:16148061 ]

Associated OMIM IDs

203750 (Beta-ketothiolase deficiency)
606054 (Propionic acidemia)
300438 (2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB022337

KNApSAcK ID

Not Available

Chemspider ID

4945715

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

4246

PubChem Compound

6441567

PDB ID

Not Available

ChEBI ID

73018

Food Biomarker Ontology

Not Available

VMH ID

Not Available

MarkerDB ID

MDB00000292

Good Scents ID

Not Available

References

Synthesis Reference

Riordan, James M.; Stammer, Charles H. Synthesis of unsaturated azlactones from N-acylamino acids. Journal of Organic Chemistry (1974), 39(5), 654-9.; Carter, S. M. Bonham; Watson, D. G.; Midgley, J. M.; Logan, R. W. Synthesis and characterization of acyl glycines. Their measurement in single blood spots by gas chromatography-mass spectrometry to diagnose inborn errors of metabolism. Journal of Chromatography, B: Biomedical Applications (1996), 677(1), 29-35.

Material Safety Data Sheet (MSDS)

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General References

Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. [PubMed:8087979 ]
Aramaki S, Lehotay D, Sweetman L, Nyhan WL, Winter SC, Middleton B: Urinary excretion of 2-methylacetoacetate, 2-methyl-3-hydroxybutyrate and tiglylglycine after isoleucine loading in the diagnosis of 2-methylacetoacetyl-CoA thiolase deficiency. J Inherit Metab Dis. 1991;14(1):63-74. [PubMed:1861461 ]
Tein I, Haslam RH, Rhead WJ, Bennett MJ, Becker LE, Vockley J: Short-chain acyl-CoA dehydrogenase deficiency: a cause of ophthalmoplegia and multicore myopathy. Neurology. 1999 Jan 15;52(2):366-72. [PubMed:9932958 ]
Apostoli P, Sarnico M, Bavazzano P, Bartoli D: Arsenic and porphyrins. Am J Ind Med. 2002 Sep;42(3):180-7. [PubMed:12210687 ]
Hagen T, Korson MS, Sakamoto M, Evans JE: A GC/MS/MS screening method for multiple organic acidemias from urine specimens. Clin Chim Acta. 1999 May;283(1-2):77-88. [PubMed:10404733 ]
Poll-The BT, Wanders RJ, Ruiter JP, Ofman R, Majoie CB, Barth PG, Duran M: Spastic diplegia and periventricular white matter abnormalities in 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency, a defect of isoleucine metabolism: differential diagnosis with hypoxic-ischemic brain diseases. Mol Genet Metab. 2004 Apr;81(4):295-9. [PubMed:15059617 ]
Fukao T, Zhang GX, Sakura N, Kubo T, Yamaga H, Hazama A, Kohno Y, Matsuo N, Kondo M, Yamaguchi S, Shigematsu Y, Kondo N: The mitochondrial acetoacetyl-CoA thiolase (T2) deficiency in Japanese patients: urinary organic acid and blood acylcarnitine profiles under stable conditions have subtle abnormalities in T2-deficient patients with some residual T2 activity. J Inherit Metab Dis. 2003;26(5):423-31. [PubMed:14518824 ]
Zschocke J, Ruiter JP, Brand J, Lindner M, Hoffmann GF, Wanders RJ, Mayatepek E: Progressive infantile neurodegeneration caused by 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency: a novel inborn error of branched-chain fatty acid and isoleucine metabolism. Pediatr Res. 2000 Dec;48(6):852-5. [PubMed:11102558 ]
Bennett MJ, Powell S, Swartling DJ, Gibson KM: Tiglylglycine excreted in urine in disorders of isoleucine metabolism and the respiratory chain measured by stable isotope dilution GC-MS. Clin Chem. 1994 Oct;40(10):1879-83. [PubMed:7923765 ]
Fukao T, Kodama A, Aoyanagi N, Tsukino R, Uemura S, Song XQ, Watanebe H, Kuhara T, Matsumoto I, Orii T, Kondo N: Mild form of beta-ketothiolase deficiency (mitochondrial acetoacetyl-CoA thiolase deficiency) in two Japanese siblings: identification of detectable residual activity and cross-reactive material in EB-transformed lymphocytes. Clin Genet. 1996 Oct;50(4):263-6. [PubMed:9001814 ]

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 Tiglylglycine (HMDB0000959)

MOL for HMDB0000959 (Tiglylglycine)

3D MOL for HMDB0000959 (Tiglylglycine)

3D SDF for HMDB0000959 (Tiglylglycine)

3D-SDF for HMDB0000959 (Tiglylglycine)

PDB for HMDB0000959 (Tiglylglycine)

3D PDB for HMDB0000959 (Tiglylglycine)

SMILES for HMDB0000959 (Tiglylglycine)

INCHI for HMDB0000959 (Tiglylglycine)

Structure for HMDB0000959 (Tiglylglycine)

3D Structure for HMDB0000959 (Tiglylglycine)

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