MiMeDB: Showing metabocard for 3-Methylglutaconic acid (MMDBc0000522)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2020-12-10 18:39:41 UTC

Update Date

2024-04-30 19:32:47 UTC

Metabolite ID

MMDBc0000522

Metabolite Identification

Common Name

3-Methylglutaconic acid

Description

3-Methylglutaconic acid is an intermediate (as the CoA thioester) in the leucine degradative pathway as well as the mevalonate shunt, a pathway that links isoprenoid metabolism with mitochondrial acetyl-CoA metabolism (PMID: 7603789 ). 3-methylglutaconyl-CoA hydratase is involved in the metabolism process of 3-methylglutaconic acid. When present in sufficiently high levels, 3-methylglutaconic acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of 3-methylglutaconic acid are associated with at least five inborn errors of metabolism including 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, 3-methylglutaconic aciduria type I, 3-methylglutaconic aciduria type III, 3-methylglutaconic aciduria type IV, and guanidinoacetate methyltransferase deficiency (GAMT deficiency). 3-Methylglutaconic acid is an organic acid. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of the untreated IEMs mentioned above. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures.

Structure

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

Synonyms

Value	Source
3-Methylglutaconate	Generator
b-Methylglutaconate	HMDB
b-Methylglutaconic acid	HMDB
beta-Methylglutaconate	HMDB
beta-Methylglutaconic acid	HMDB
Β-methylglutaconic acid	HMDB
3-Methyl-2-pentenedioic acid	HMDB
3E-Methylglutaconate	HMDB
3-Methylglutaconic acid	HMDB, MeSH

Molecular Formula

C₆H₈O₄

Average Mass

144.1253

Monoisotopic Mass

144.042258744

IUPAC Name

(2E)-3-methylpent-2-enedioic acid

Traditional Name

β-methylglutaconic acid

CAS Registry Number

Not Available

SMILES

C\C(CC(O)=O)=C/C(O)=O

InChI Identifier

InChI=1S/C6H8O4/c1-4(2-5(7)8)3-6(9)10/h2H,3H2,1H3,(H,7,8)(H,9,10)/b4-2+

InChI Key

WKRBKYFIJPGYQC-DUXPYHPUSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as methyl-branched fatty acids. These are fatty acids with an acyl chain that has a methyl branch. Usually, they are saturated and contain only one or more methyl group. However, branches other than methyl may be present.

Kingdom

Organic compounds

Super Class

Lipids and lipid-like molecules

Class

Fatty Acyls

Sub Class

Fatty acids and conjugates

Direct Parent

Methyl-branched fatty acids

Alternative Parents

Substituents

Methyl-branched fatty acid
Unsaturated fatty acid
Dicarboxylic acid or derivatives
Carboxylic acid
Carboxylic acid derivative
Organic oxygen compound
Organic oxide
Hydrocarbon derivative
Organooxygen compound
Carbonyl group
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

dicarboxylic acid (CHEBI:37245 )
Dicarboxylic acids (LMFA01170068 )

Functional Ontology

Not Available

Physical Properties

State

Expected Solid

Predicted Properties

Property	Value	Source
Water Solubility	8.69 g/L	ALOGPS
logP	0.37	ALOGPS
logP	0.29	ChemAxon
logS	-1.2	ALOGPS
pKa (Strongest Acidic)	3.85	ChemAxon
Physiological Charge	-2	ChemAxon
Hydrogen Acceptor Count	4	ChemAxon
Hydrogen Donor Count	2	ChemAxon
Polar Surface Area	74.6 Å²	ChemAxon
Rotatable Bond Count	3	ChemAxon
Refractivity	33.51 m³·mol⁻¹	ChemAxon
Polarizability	13.27 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Spectra

Spectrum Type	Description	Splash Key	Deposition Date	View
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-0015-9200000000-ff306cede85d252e0d4c	2017-09-01	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positive	splash10-00di-9340000000-0b493822a103307b8f66	2017-10-06	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_2_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 40V, Negative	splash10-000f-9000000000-fa6952af79fc3b2759fe	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 20V, Negative	splash10-0a4j-9000000000-3a5a017393a0592a65ff	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 40V, Negative	splash10-000f-9000000000-14ef6163925508d3f666	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Negative	splash10-0002-9000000000-ada755597943f74819c4	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Negative	splash10-0002-9000000000-70f10348ac9e84418765	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 20V, Negative	splash10-0a4i-9000000000-5a9ee69bdb3960321fa6	2021-09-20	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-002b-2900000000-b6862aeffb1aa9fae1d0	2016-08-03	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-000i-9300000000-7ab01dfb0b86abdac817	2016-08-03	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-0006-9000000000-3692ceff17a888ae8836	2016-08-03	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-0006-5900000000-dc4d4a80a164f3fd1fea	2016-08-03	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-0007-8900000000-7ece848c8011e4a20200	2016-08-03	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-0a7l-9300000000-8b54483c15a17429b9f8	2016-08-03	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-0002-9200000000-70569e6181007374644c	2021-09-24	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-000t-9000000000-9426c88487da5c337c1b	2021-09-24	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-000x-9000000000-60467181d27d878d6aca	2021-09-24	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-0002-9400000000-79724b8b1198fb442949	2021-09-24	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-001j-9000000000-4f399595435fe16b3880	2021-09-24	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-001c-9000000000-1ee161ce72a30f1b0814	2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 100 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 100 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 1000 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 200 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 200 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 300 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 300 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 400 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 400 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 500 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 600 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 700 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 700 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 800 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 800 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 900 MHz, H₂O, predicted)		2022-08-18	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 900 MHz, H₂O, predicted)		2022-08-18	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm
Extracellular
Membrane

Biospecimen Locations

Blood
Cerebrospinal Fluid (CSF)
Feces
Placenta
Urine

Tissue Locations

Placenta

Associated OMIM IDs

114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)

Human Proteins and Enzymes

Protein ID	Protein Name	Protein Type	Uniprot ID	Species

Human Pathways

Pathways

Name	SMPDB/PathBank

Metabolic Reactions

Reactions

Reaction ID	Precursor	Product	Enzyme	Reaction Type	Data Source	Reference
MMDBr0008592	3-Methylglutaconyl-CoA	3-Methylglutaconic acid	Not Available		VMH	30371894
MMDBr0008596	3-Methylglutaconic acid	3-Methylglutaric acid	Not Available		VMH	30371894

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Evidence Type	Host and Biospecimen	Data Source	Reference
3-hydroxy-3-methylglutaryl-coa lyase deficiency	Increased	Association	Human Urine	HMDB	15505778
3-hydroxy-3-methylglutaryl-coa lyase deficiency		Association	Human Urine	HMDB	23705938
3-methylglutaconic aciduria type i	Increased	Association	Human Cerebrospinal Fluid (CSF)	HMDB	16541463
3-methylglutaconic aciduria type i	Increased	Association	Human Urine	HMDB	20855850
3-methylglutaconic aciduria type i	Increased	Association	Human Urine	HMDB	16541463
3-methylglutaconic aciduria type ii, x-linked	Slight Increase	Association	Human Urine	HMDB	10484795
3-methylglutaconic aciduria type v		Association	Human Urine	HMDB	22797137
Eosinophilic esophagitis	Slight Decrease	Association	Human Urine	HMDB	Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Eosinophilic esophagitis	Slight Increase	Association	Human Urine	HMDB	Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
3-hydroxy-3-methylglutaryl-coa lyase deficiency	Slight Decrease	Association	Human Urine	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
3-hydroxy-3-methylglutaryl-coa lyase deficiency		Association	Human Urine	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
3-methylglutaconic aciduria type i		Association	Human Urine	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)

Microbial Sources

Superkingdom/Kingdom	Phylum	Total species	Hosts and Body Sites	Microbial Links
Bacteria	Firmicutes	1	Human stool; Human ; Human feces	Indirect association between microbiota composition and metabolites measured in host biospecimen
Archaea	Euryarchaeota	1	Human feces	Indirect association between microbiota composition and metabolites measured in host biospecimen
Archaea	Euryarchaeota	1	Human feces; Human	Indirect association between microbiota composition and metabolites measured in host biospecimen

Exposure Sources

Host Biospecimen and Location

Host and Biospecimen	Status	Mean	Std	Min	Max	Units	Age	Sex	Health Condition	Data Source	Reference
Human Feces	Detected but not Quantified									HMDB
Human Urine	Detected and Quantified	3714				umol/mmol creatinine	Adult (>18 years old)	Female	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	HMDB	15505778
Human Urine	Detected and Quantified	3714				umol/mmol creatinine	Adult (>18 years old)	Female	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	HMDB	15505778
Human Urine	Detected and Quantified	4166.4				umol/mmol creatinine	Newborn (0-30 days old)	Male	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	HMDB	23705938
Human Cerebrospinal fluid (csf)	Detected and Quantified	125				uM	Adult (>18 years old)	Female	3-methylglutaconic aciduria type I	HMDB	16541463
Human Urine	Detected and Quantified	570				umol/mmol creatinine	Children (1-13 years old)	Not Specified	3-Methylglutaconic Aciduria (TYPE I)	HMDB	20855850
Human Urine	Detected and Quantified			32	51	umol/mmol creatinine	Adult (>18 years old)	Female	3-methylglutaconic aciduria type I	HMDB	16541463
Human Urine	Detected and Quantified			62	90	umol/mmol creatinine	Adult (>18 years old)	Female	3-methylglutaconic aciduria type I	HMDB	16541463
Human Urine	Detected and Quantified			94	141	umol/mmol creatinine	Adult (>18 years old)	Not Specified	3-Methylglutaconic Aciduria (TYPE I)	HMDB	20855850
Human Urine	Detected and Quantified			0	230	umol/mmol creatinine	Infant (0-1 year old)	Male	3-methylglutaconic aciduria type II, X-linked	HMDB	10484795
Human Urine	Detected and Quantified			16	68	umol/mmol creatinine	Adolescent (13-18 years old)	Female	3-Methylglutaconic Aciduria (Type IV)	HMDB	19015156
Human Urine	Detected and Quantified			4	200	umol/mmol creatinine	Children (1-13 years old)	Both	3-Methylglutaconic Aciduria (Type IV)	HMDB	19015156
Human Urine	Detected and Quantified	13000				umol/mmol creatinine	Children (1-13 years old)	Male	3-Methylglutaconic Aciduria (Type V)	HMDB	22797137
Human Urine	Detected and Quantified			0.0160	0.196	umol/mmol creatinine	Children (1-13 years old)	Female	3-Methylglutaconic Aciduria (Type VI)	HMDB	16527507
Human Urine	Detected and Quantified			29	109	umol/mmol creatinine	Children (1-13 years old)	Female	3-Methylglutaconic Aciduria (Type VII)	HMDB	25597510
Human Urine	Detected and Quantified			34	160	umol/mmol creatinine	Adolescent (13-18 years old)	Female	3-Methylglutaconic Aciduria (Type VII)	HMDB	25597510
Human Urine	Detected and Quantified			52	93	umol/mmol creatinine	Infant (0-1 year old)	Female	3-Methylglutaconic Aciduria (Type VII)	HMDB	25597510
Human Urine	Detected and Quantified			20	488	umol/mmol creatinine	Children (1-13 years old)	Both	Sengers syndrome	HMDB	16736096
Human Urine	Detected and Quantified	<0.0200				umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	16527507
Human Cerebrospinal fluid (csf)	Detected and Quantified	<10				uM	Adult (>18 years old)	Female	Normal	HMDB	16541463
Human Urine	Detected and Quantified	<10				umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	10484795
Human Urine	Detected and Quantified	<12				umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	25597510
Human Urine	Detected and Quantified	<1500				umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	22797137
Human Urine	Detected and Quantified	<20				umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	19015156
Human Urine	Detected and Quantified	<20				umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	HMDB	25597510
Human Urine	Detected and Quantified	<25				umol/mmol creatinine	Adult (>18 years old)	Female	Normal	HMDB	15505778
Human Urine	Detected and Quantified	<25				umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	15505778
Human Urine	Detected and Quantified	<6				umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	16736096
Human Urine	Detected and Quantified	<6				umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	23705938
Human Urine	Detected and Quantified			0	0.2	umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	HMDB	31963255
Human Urine	Detected and Quantified			0	26	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	31506554
Human Urine	Detected and Quantified			0	4	umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	31963255
Human Urine	Detected and Quantified			0	6.0	umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	31963255
Human Urine	Detected and Quantified			1.0	6.5	umol/mmol creatinine	Adult (>18 years old)	Female	Normal	HMDB	16541463
Human Urine	Detected and Quantified			1.8	7.6	umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	HMDB	31963255
Human Urine	Detected and Quantified	6.2		2.8	8.3	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected and Quantified	7.5		3.4	9.5	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected and Quantified	10.714	4.929			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	14.626	7.998			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	4.5		0.00	9.0	umol/mmol creatinine	Adult (>18 years old)	Both	3-Hydroxy-3-methylglutaryl-CoA lyase deficiency	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	5070.0		140.0	10000.0	umol/mmol creatinine	Children (1-13 years old)	Both	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	750.0		500.0	1000.0	umol/mmol creatinine	Children (1-13 years old)	Both	3-Methylglutaconic Aciduria (TYPE I)	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	10.707	4.243			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	15.86	6.052			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	<12.42				umol/mmol creatinine	Children (1 - 18 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Placenta	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	27543620
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	29808030
Human Blood	Detected but not Quantified									HMDB

External Links

HMDB ID

HMDB0000522

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB022090

KNApSAcK ID

C00052150

Chemspider ID

1267861

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

3-Methylglutaconic acid

METLIN ID

5507

PubChem Compound

1551553

PDB ID

Not Available

ChEBI ID

37245

Food Biomarker Ontology

Not Available

References

Synthesis Reference

Not Available

General References

Holme E, Greter J, Jacobson CE, Larsson NG, Lindstedt S, Nilsson KO, Oldfors A, Tulinius M: Mitochondrial ATP-synthase deficiency in a child with 3-methylglutaconic aciduria. Pediatr Res. 1992 Dec;32(6):731-5. doi: 10.1203/00006450-199212000-00022. [PubMed:1287564 ]
Sheffer RN, Zlotogora J, Elpeleg ON, Raz J, Ben-Ezra D: Behr's syndrome and 3-methylglutaconic aciduria. Am J Ophthalmol. 1992 Oct 15;114(4):494-7. doi: 10.1016/s0002-9394(14)71864-1. [PubMed:1384336 ]
Mitchell GA, Jakobs C, Gibson KM, Robert MF, Burlina A, Dionisi-Vici C, Dallaire L: Molecular prenatal diagnosis of 3-hydroxy-3-methylglutaryl CoA lyase deficiency. Prenat Diagn. 1995 Aug;15(8):725-9. doi: 10.1002/pd.1970150807. [PubMed:7479590 ]
Kelley RI, Kratz L: 3-methylglutaconic acidemia in Smith-Lemli-Opitz syndrome. Pediatr Res. 1995 May;37(5):671-4. doi: 10.1203/00006450-199505000-00020. [PubMed:7603789 ]
Kelley RI: Quantification of 3-methylglutaconic acid in urine, plasma, and amniotic fluid by isotope-dilution gas chromatography/mass spectrometry. Clin Chim Acta. 1993 Nov 15;220(2):157-64. doi: 10.1016/0009-8981(93)90044-5. [PubMed:8111960 ]
Costeff H, Elpeleg O, Apter N, Divry P, Gadoth N: 3-Methylglutaconic aciduria in "optic atrophy plus". Ann Neurol. 1993 Jan;33(1):103-4. doi: 10.1002/ana.410330117. [PubMed:8494328 ]
Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

Showing metabocard for 3-Methylglutaconic acid (MMDBc0000522)

MOL for #<Metabolite:0x00007fdb3cf93b38>

3D MOL for #<Metabolite:0x00007fdb3cf93b38>

3D SDF for #<Metabolite:0x00007fdb3cf93b38>

3D-SDF for #<Metabolite:0x00007fdb3cf93b38>

PDB for #<Metabolite:0x00007fdb3cf93b38>

3D PDB for #<Metabolite:0x00007fdb3cf93b38>

SMILES for #<Metabolite:0x00007fdb3cf93b38>

INCHI for #<Metabolite:0x00007fdb3cf93b38>

Structure for #<Metabolite:0x00007fdb3cf93b38>

3D Structure for #<Metabolite:0x00007fdb3cf93b38>