Livestock Metabolome Database: Showing metabocard for 3-Hydroxymethylglutaric acid (LMDB00143)

Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References XML

Record Information

Version

1.0

Creation Date

2016-07-13 19:43:18 UTC

Update Date

2016-08-06 20:09:00 UTC

Lmdb

LMDB00143

Secondary Accession Numbers

None

Metabolite Identification

Common Name

3-Hydroxymethylglutaric acid

Description

3-Hydroxymethylglutaric acid is a metabolite that accumulates in the urine of patients affected by 3-Hydroxy-3-methylglutaric aciduria, a rare inborn error of metabolism (OMIM 246450 ). 3-Hydroxy-3-methylglutaric aciduria is caused by reduced enzyme activity of the intramitochondrial 3-hydroxy-3-methylglutaryl-CoA lyase (EC 4.1.3.4), the enzyme that catalyzes the final step of leucine degradation and plays a key role in ketone body formation. The profile of urinary organic acids is different from that of the other identified defects of leucine degradation--maple syrup urine disease (OMIM 248600 ), isovaleric acidemia (OMIM 243500 ), and methylcrotonylglycinemia (OMIM 210200 ). Clinical manifestations include hepatomegaly, lethargy or coma and apnoea. Biochemically there is a characteristic absence of ketosis with hypoglycemia, acidosis, hipertransaminasemia and variable hyperammoniemia. The urinary organic acid profile includes elevated concentrations of 3-hydroxy-3-isovaleric, 3-hydroxy-3-methylglutaric, 3-methylglutaconic and 3-methylglutaric acids. (PMID: 10916782 , 9658458 , 3063529 ).

Structure

MOL SDF 3D-SDF PDB SMILES InChI View 3D Structure

Synonyms

Value	Source
(S)-3-Hydroxy-3-methylglutaric acid	ChEBI
(S)-Meglutol	ChEBI
3-HYDROXY-3-methyl-glutarIC ACID	ChEBI
3-Hydroxy-3-methylpentanedioic acid	ChEBI
beta-Hydroxy-beta-methylglutaric acid	ChEBI
Dicrotalic acid	ChEBI
Meglutol	ChEBI
Meglutolum	ChEBI
3-Hydorxy-3-methylglutaric acid	Kegg
(S)-3-Hydroxy-3-methylglutarate	Generator
3-HYDROXY-3-methyl-glutarate	Generator
3-Hydroxy-3-methylpentanedioate	Generator
b-Hydroxy-b-methylglutarate	Generator
b-Hydroxy-b-methylglutaric acid	Generator
beta-Hydroxy-beta-methylglutarate	Generator
Β-hydroxy-β-methylglutarate	Generator
Β-hydroxy-β-methylglutaric acid	Generator
Dicrotalate	Generator
3-Hydorxy-3-methylglutarate	Generator
3-Hydroxymethylglutarate	Generator
3-Hydroxy-3-methylglutarate	HMDB
3-Hydroxy-3-methylglutaric acid	HMDB
3-Methyl-3-hydroxyglutarate	HMDB
3-Methyl-3-hydroxyglutaric acid	HMDB
CB 337	HMDB
3 Hydroxy 3 methylpentanedioic acid	HMDB
Acid, 3-hydroxy-3-methylglutaric	HMDB
Acid, 3-hydroxy-3-methylpentanedioic	HMDB
beta Hydroxy beta methylglutarate	HMDB
3 Hydroxy 3 methylglutaric acid	HMDB
3-Methyl-3-hydroxypentanedioate	HMDB
3-Methyl-3-hydroxypentanedioic acid	HMDB
HMG	HMDB
HMGA	HMDB
Lipoglutaren	HMDB
Medroglutarate	HMDB
Medroglutaric acid	HMDB

Chemical Formula

C₆H₁₀O₅

Average Molecular Weight

162.1406

Monoisotopic Molecular Weight

162.05282343

IUPAC Name

3-hydroxy-3-methylpentanedioic acid

Traditional Name

meglutol

CAS Registry Number

503-49-1

SMILES

CC(O)(CC(O)=O)CC(O)=O

InChI Identifier

InChI=1S/C6H10O5/c1-6(11,2-4(7)8)3-5(9)10/h11H,2-3H2,1H3,(H,7,8)(H,9,10)

InChI Key

NPOAOTPXWNWTSH-UHFFFAOYSA-N

Chemical Taxonomy

Description

belongs to the class of organic compounds known as hydroxy fatty acids. These are fatty acids in which the chain bears a hydroxyl group.

Kingdom

Organic compounds

Super Class

Lipids and lipid-like molecules

Class

Fatty Acyls

Sub Class

Fatty acids and conjugates

Direct Parent

Hydroxy fatty acids

Alternative Parents

Substituents

Methyl-branched fatty acid
Short-chain hydroxy acid
Hydroxy fatty acid
Branched fatty acid
Dicarboxylic acid or derivatives
Tertiary alcohol
Carboxylic acid
Carboxylic acid derivative
Organic oxygen compound
Organic oxide
Hydrocarbon derivative
Organooxygen compound
Carbonyl group
Alcohol
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

tertiary alcohol (CHEBI:16831 )
dicarboxylic acid (CHEBI:16831 )
3-hydroxy carboxylic acid (CHEBI:16831 )

Ontology

Status

Detected but not Quantified

Origin

Not Available

Biofunction

Not Available

Application

Not Available

Cellular locations

Not Available

Physical Properties

State

Not Available

Experimental 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

Predicted Properties

Property	Value	Source
logP	-0.88	ALOGPS
logP	-0.75	ChemAxon
logS	0.23	ALOGPS
pKa (Strongest Acidic)	3.68	ChemAxon
pKa (Strongest Basic)	-3	ChemAxon
Physiological Charge	-2	ChemAxon
Hydrogen Acceptor Count	5	ChemAxon
Hydrogen Donor Count	3	ChemAxon
Polar Surface Area	94.83 Å²	ChemAxon
Rotatable Bond Count	4	ChemAxon
Refractivity	34.14 m³·mol⁻¹	ChemAxon
Polarizability	14.55 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	Yes	ChemAxon
Veber's Rule	Yes	ChemAxon
MDDR-like Rule	Yes	ChemAxon

Spectra

Spectrum Type	Description	Splash Key	View
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)	splash10-0002-0930000000-67cdb23f1a51498bb80d	Spectrum
GC-MS	GC-MS Spectrum - GC-MS (3 TMS)	splash10-05nb-2950000000-10da019551eceff729ec	Spectrum
GC-MS	GC-MS Spectrum - EI-B (Non-derivatized)	splash10-0002-0960000000-75a1f0bc3ffb60117676	Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0002-0930000000-67cdb23f1a51498bb80d	Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-05nb-2950000000-10da019551eceff729ec	Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0002-0920000000-77008c8c7fb6eb798e79	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-0udi-4900000000-ae0329f185525bab6028	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (3 TMS) - 70eV, Positive	splash10-0079-9253000000-af0bf7551c9147193ed4	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_1) - 70eV, Positive	Not Available	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_2) - 70eV, Positive	Not Available	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_1) - 70eV, Positive	Not Available	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_2) - 70eV, Positive	Not Available	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_2_1) - 70eV, Positive	Not Available	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_2_2) - 70eV, Positive	Not Available	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_3_1) - 70eV, Positive	Not Available	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)	splash10-05fr-2900000000-d5b3d7ccc3617f392a63	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)	splash10-004i-9800000000-1a510972071bf02a23f7	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)	splash10-0kdi-9300000000-6afee652d0eb18e589c3	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative	splash10-03di-0900000000-ae679b03ae8cdbc1cc40	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative	splash10-0002-9300000000-8d7565d3b9cbed5d13ac	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative	splash10-0a4i-9000000000-d06ad8ce44567793a49d	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative	splash10-0a4i-9000000000-f3c4c2c0cb3ff49db482	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative	splash10-0a4i-9000000000-98c0337e0bebe4fefe6c	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positive	splash10-01qa-2900000000-182405b2867309ab7df8	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative	splash10-03dj-5900000000-d9ffa79e6b62296f2626	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-03di-0900000000-2847ca367167b7cf107b	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-0002-9300000000-f9ab44912911fd1e9ec7	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-0a4i-9000000000-589bf77ae5f575911b64	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-0a4i-9000000000-f3c4c2c0cb3ff49db482	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-0a4i-9000000000-98c0337e0bebe4fefe6c	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF , negative	splash10-03dj-5900000000-d9ffa79e6b62296f2626	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF , positive	splash10-01qa-2900000000-182405b2867309ab7df8	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 20V, Negative	splash10-0a4i-9000000000-f94de43a0b43873232bf	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Negative	splash10-0a4j-9300000000-9861bfd5df213d5b52e4	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-0002-0900000000-a3631689983e6d6a3be7	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-002b-2900000000-54e16eaede819dea2bdc	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-000i-9600000000-31a4e319505eebd3d1d1	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-03xr-2900000000-405383db70ec0564b916	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-07bg-5900000000-dfc057320722ae1d926c	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-0a4m-9300000000-e5a720c14cbc5de82d4d	Spectrum
1D NMR	1H NMR Spectrum	Not Available	Spectrum
2D NMR	[1H,13C] 2D NMR Spectrum	Not Available	Spectrum

Biological Properties

Cellular Locations

Not Available

Biofluid Locations and Tissue Locations

Plasma
Urine

Concentrations

Biofluid	Status	Value	Condition	Species	Reference	Details
Plasma	Detected but not Quantified	Not Applicable	Not Available	Bovine	24070026	details
Urine	Detected but not Quantified	Not Applicable	Not Available	Porcine	24696864	details

External Links

DrugBank ID

DB04377

HMDB ID

HMDB0000355

FooDB ID

FDB004207

Phenol Explorer ID

Not Available

KNApSAcK ID

C00001187

BiGG ID

Not Available

BioCyc ID

CPD-547

METLIN ID

5344

PDB ID

Not Available

Wikipedia Link

Not Available

Chemspider ID

ChEBI ID

PubChem Compound ID

Kegg Compound ID

YMDB ID

Not Available

ECMDB ID

Not Available

References

Synthesis Reference

Not Available

Material Safety Data Sheet (MSDS)

Not Available

General References

Koling S, Kalhoff H, Schauerte P, Lehnert W, Diekmann L: [3-hydroxy-3-methylglutaraciduria (case report of a female Turkish sisters with 3-hydroxy-3- methylglutaryl-Coenzyme A lyase deficiency]. Klin Padiatr. 2000 May-Jun;212(3):113-6. [10916782 ]
Eiris J, Ribes A, Fernandez-Prieto R, Rodriguez-Garcia J, Rodriguez-Segade S, Castro-Gago M: [3-hydroxy-3-methylglutaric aciduria and recurrent Reye-like syndrome]. Rev Neurol. 1998 Jun;26(154):911-4. [9658458 ]
Gibson KM, Breuer J, Nyhan WL: 3-Hydroxy-3-methylglutaryl-coenzyme A lyase deficiency: review of 18 reported patients. Eur J Pediatr. 1988 Dec;148(3):180-6. [3063529 ]

Showing metabocard for 3-Hydroxymethylglutaric acid (LMDB00143)

Structure for LMDB00143 (3-Hydroxymethylglutaric acid)