MiMeDB: Showing metabocard for Methylmalonic acid (MMDBc0000502)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2020-12-10 18:39:13 UTC

Update Date

2024-10-11 00:44:49 UTC

Metabolite ID

MMDBc0000502

Metabolite Identification

Common Name

Methylmalonic acid

Description

Methylmalonic acid is a malonic acid derivative, which is a vital intermediate in the metabolism of fat and protein. In particular, the coenzyme A-linked form of methylmalonic acid, methylmalonyl-CoA, is converted into succinyl-CoA by methylmalonyl-CoA mutase in a reaction that requires vitamin B12 as a cofactor. In this way, methylmalonic acid enters the Krebs cycle and is thus part of one of the anaplerotic reactions. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This inborn error of metabolism is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA. Methylmalonic acid is also found to be associated with other inborn errors of metabolism, including cobalamin deficiency, cobalamin malabsorption, malonyl-CoA decarboxylase deficiency, and transcobalamin II deficiency. When present in sufficiently high levels, methylmalonic 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 methylmalonic acid are associated with at least 5 inborn errors of metabolism, including Malonyl CoA decarboxylase deficiency, Malonic Aciduria, Methylmalonate Semialdehyde Dehydrogenase Deficiency, Methylmalonic Aciduria and Methylmalonic Aciduria Due to Cobalamin-Related Disorders. Methylmalonic acid is an organic acid and 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 abnormalities, kidney abnormalities, liver damage, 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
1,1-Ethanedicarboxylic acid	ChEBI
2-Methylmalonic acid	ChEBI
alpha-Methylmalonic acid	ChEBI
Isosuccinic acid	ChEBI
1,1-Ethanedicarboxylate	Generator
2-Methylmalonate	Generator
a-Methylmalonate	Generator
a-Methylmalonic acid	Generator
alpha-Methylmalonate	Generator
Α-methylmalonate	Generator
Α-methylmalonic acid	Generator
Isosuccinate	Generator
Methylmalonate	Generator
Methyl-malonate	HMDB
Methyl-malonic acid	HMDB
Methyl-propanedioate	HMDB
Methyl-propanedioic acid	HMDB
Methylpropanedioate	HMDB
Methylpropanedioic acid	HMDB
Acid, methylmalonic	HMDB
Methylmalonic acid	KEGG

Molecular Formula

C₄H₆O₄

Average Mass

118.088

Monoisotopic Mass

118.02660868

IUPAC Name

2-methylpropanedioic acid

Traditional Name

methylmalonic acid

CAS Registry Number

Not Available

SMILES

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

InChI Identifier

InChI=1S/C4H6O4/c1-2(3(5)6)4(7)8/h2H,1H3,(H,5,6)(H,7,8)

InChI Key

ZIYVHBGGAOATLY-UHFFFAOYSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as dicarboxylic acids and derivatives. These are organic compounds containing exactly two carboxylic acid groups.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Dicarboxylic acids and derivatives

Direct Parent

Dicarboxylic acids and derivatives

Alternative Parents

Substituents

1,3-dicarbonyl compound
Dicarboxylic acid or derivatives
Carboxylic acid
Organic oxygen compound
Organic oxide
Hydrocarbon derivative
Organooxygen compound
Carbonyl group
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

C4-dicarboxylic acid (CHEBI:30860 )
Dicarboxylic acids (LMFA01170118 )

Functional Ontology

Not Available

Physical Properties

State

Expected Solid

Predicted Properties

Property	Value	Source
Water Solubility	149 g/L	ALOGPS
logP	0.17	ALOGPS
logP	0.21	ChemAxon
logS	0.1	ALOGPS
pKa (Strongest Acidic)	2.48	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	2	ChemAxon
Refractivity	23.56 m³·mol⁻¹	ChemAxon
Polarizability	10.06 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

Not Available

Biological Properties

Cellular Locations

Cytoplasm

Biospecimen Locations

Blood
Cerebrospinal Fluid (CSF)
Feces
Kidney
Liver
Placenta
Urine

Tissue Locations

Kidney
Liver
Placenta

Associated OMIM IDs

114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)

Human Proteins and Enzymes

Protein ID	Protein Name	Protein Type	Uniprot ID	Species
MMDBp0191797	Aldehyde oxidase	Unknown	Q06278	Homo sapiens
MMDBp0191961	4-trimethylaminobutyraldehyde dehydrogenase	Unknown	P49189	Homo sapiens
MMDBp0191963	Alpha-aminoadipic semialdehyde dehydrogenase	Unknown	P49419	Homo sapiens
MMDBp0191964	Aldehyde dehydrogenase family 1 member A3	Unknown	P47895	Homo sapiens
MMDBp0191965	Aldehyde dehydrogenase, mitochondrial	Unknown	P05091	Homo sapiens
MMDBp0191966	Fatty aldehyde dehydrogenase	Unknown	P51648	Homo sapiens
MMDBp0191967	Aldehyde dehydrogenase X, mitochondrial	Unknown	P30837	Homo sapiens

Human Pathways

Pathways

Name	SMPDB/PathBank

Metabolic Reactions

Reactions

Reaction ID	Precursor	Product	Enzyme	Reaction Type	Data Source	Reference
MMDBr0038727	R-Methylmalonyl-CoA	Methylmalonic acid	Thioesterase TesA	Hydrolysis	RHEA	34755880
MMDBr0038729	(S)-Methylmalonyl-CoA	Methylmalonic acid	Thioesterase TesA	Hydrolysis	RHEA	34755880
MMDBr0038777	(R)-Methylmalonyl-CoA	Methylmalonic acid	Thioesterase TesA	Hydrolysis	RHEA	34755880
MMDBr0038782	Methylmalonyl-CoA	Methylmalonic acid	Thioesterase TesA	Hydrolysis	RHEA	34755880

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Evidence Type	Host and Biospecimen	Data Source	Reference
Alzheimer's disease		Association	Human Cerebrospinal Fluid (CSF)	HMDB	16227558
Alzheimer's disease	Increased	Association	Human Blood	HMDB	8356878
Fumarase deficiency	Increased	Association	Human Urine	HMDB	26078636
Malonyl-coa decarboxylase deficiency	Increased	Association	Human Blood	HMDB	24613099
Malonyl-coa decarboxylase deficiency	Increased	Association	Human Cerebrospinal Fluid (CSF)	HMDB	24613099
Malonyl-coa decarboxylase deficiency	Increased	Association	Human Urine	HMDB	3709568
Propionic acidemia		Association	Human Urine	HMDB	28853722
Transcobalamin ii deficiency	Increased	Association	Human Urine	HMDB	10518276
Vitamin b12 deficiency	Increased	Association	Human Blood	HMDB	3700655
Vitamin b12 deficiency	Increased	Association	Human Blood	HMDB	8412012
Eosinophilic esophagitis	Increased	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	Increased	Association	Human Urine	HMDB	Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Cobalamin malabsorption	Increased	Association	Human Urine	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Cobalamin malabsorption	Increased	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/Eubacteria	Firmicutes	80	Human feces; Human ; Human subgingival plaque	Indirect association between microbiota composition and metabolites measured in host biospecimen; Metabolic reconstruction
Bacteria/Eubacteria	Proteobacteria	34	Human feces; Human sputum; Human	Metabolic reconstruction
Bacteria/Eubacteria	Bacteroidetes	9	Human feces; Human ; Human supragingival plaque; Human subgingival plaque; Human saliva	Metabolic reconstruction
Archaea	Thaumarchaeota	1		Metabolic reconstruction
Bacteria	Firmicutes	7	Human feces; Human ; Human subgingival plaque	Indirect association between microbiota composition and metabolites measured in host biospecimen; Metabolic reconstruction
Bacteria/Eubacteria	Actinobacteria	9	Human feces; Human	Metabolic reconstruction
Archaea	Euryarchaeota	1		Metabolic reconstruction
Bacteria	Proteobacteria	1	Human feces; Human sputum; Human	Metabolic reconstruction
Eukaryota/Fungi	Ascomycota	1	Human feces; Human saliva	Unknown

Exposure Sources

Other Exposures

Source Type	Data Source	Reference
Drugs - Antimicrobial	U.S. Environmental Protection Agency	CompTox
Cosmetics	U.S. Environmental Protection Agency	CompTox
Domestics	U.S. Environmental Protection Agency	CompTox

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 Cerebrospinal fluid (csf)	Detected and Quantified	0.30	0.12			uM	Elderly (>65 years old)	Not Specified	Alzheimer's disease	HMDB	16227558
Human Blood	Detected and Quantified	0.480	0.062			uM	Adult (>18 years old)	Both	Alzheimer's disease	HMDB	8356878
Human Urine	Detected and Quantified	200				umol/mmol creatinine	Infant (0-1 year old)	Female	Cobalamin F disease (cblF)	HMDB	21910240
Human Blood	Detected and Quantified			10	15.4	uM	Adult (>18 years old)	Both	Combined malonic and methylmalonic aciduria	HMDB	21841779
Human Urine	Detected and Quantified			29.200	104.500	umol/mmol creatinine	Adult (>18 years old)	Both	Combined malonic and methylmalonic aciduria	HMDB	21841779
Human Urine	Detected and Quantified	3.83				umol/mmol creatinine	Infant (0-1 year old)	Male	Fumarase deficiency	HMDB	26078636
Human Blood	Detected and Quantified	2.8				uM	Adolescent (13-18 years old)	Female	Malonyl-CoA decarboxylase deficiency	HMDB	24613099
Human Cerebrospinal fluid (csf)	Detected and Quantified	13				uM	Adolescent (13-18 years old)	Female	Malonyl-CoA decarboxylase deficiency	HMDB	24613099
Human Urine	Detected and Quantified	33				umol/mmol creatinine	Infant (0-1 year old)	Male	Malonyl-Coa decarboxylase deficiency	HMDB	3709568
Human Blood	Detected and Quantified			1.3	4.1	uM	Children (1-13 years old)	Both	Methylmalonic aciduria mitochondrial encephelopathy Leigh-like	HMDB	17301081
Human Urine	Detected and Quantified			16	80	umol/mmol creatinine	Children (1-13 years old)	Both	Methylmalonic aciduria mitochondrial encephelopathy Leigh-like	HMDB	17301081
Human Urine	Detected and Quantified			10	100	umol/mmol creatinine	Infant (0-1 year old)	Both	Methylmalonic aciduria, cblA type	HMDB	28070528
Human Blood	Detected and Quantified			5	100	uM	Infant (0-1 year old)	Both	Methylmalonic aciduria, cblA type	HMDB	28070528
Human Urine	Detected and Quantified			10	100	umol/mmol creatinine	Infant (0-1 year old)	Both	Methylmalonic aciduria, cblB type	HMDB	28070528
Human Blood	Detected and Quantified			5	100	uM	Infant (0-1 year old)	Both	Methylmalonic aciduria, cblB type	HMDB	28070528
Human Blood	Detected and Quantified			0.8	33	uM	Children (1-13 years old)	Both	Mitochondrial encephalomyopaththy with elevanted methylmalonic acid, SUCLA2	HMDB	17287286
Human Urine	Detected and Quantified			51	212	umol/mmol creatinine	Children (1-13 years old)	Both	Mitochondrial encephalomyopaththy with elevanted methylmalonic acid, SUCLA2	HMDB	17287286
Human Urine	Detected and Quantified	0				umol/mmol creatinine	Adult (>18 years old)	Not Specified	Propionic acidemia	HMDB	28853722
Human Urine	Detected and Quantified	0.05				umol/mmol creatinine	Adult (>18 years old)	Not Specified	Propionic acidemia	HMDB	28853722
Human Urine	Detected and Quantified			126	904	umol/mmol creatinine	Newborn (0-30 days old)	Female	Transcobalamin II deficiency	HMDB	10518276
Human Blood	Detected and Quantified			0.466	188.842	uM	Adult (>18 years old)	Both	cobalamin deficiency	HMDB	3700655
Human Blood	Detected and Quantified	22.0		4.3	37.0	uM	Adult (>18 years old)	Both	Cobalamin deficiency	HMDB	8412012
Human Urine	Detected and Quantified			10	20	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	31506554
Human Urine	Detected and Quantified	<0.07				umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	28853722
Human Blood	Detected and Quantified	<0.27				uM	Infant (0-1 year old)	Both	Normal	HMDB	28070528
Human Blood	Detected and Quantified	<0.28				uM	Children (1-13 years old)	Not Specified	Normal	HMDB	17287286
Human Blood	Detected and Quantified	<0.33				uM	Children (1-13 years old)	Not Specified	Normal	HMDB	17301081
Human Blood	Detected and Quantified	<0.34				uM	Adolescent (13-18 years old)	Female	Normal	HMDB	24613099
Human Blood	Detected and Quantified	<0.4				uM	Adult (>18 years old)	Not Specified	Normal	HMDB	21841779
Human Urine	Detected and Quantified	<3				umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	HMDB	21841779
Human Urine	Detected and Quantified	<3.6				umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	17287286
Human Urine	Detected and Quantified	<4				umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	28070528
Human Urine	Detected and Quantified	<47.871				umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	26078636
Human Urine	Detected and Quantified	<5				umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	17301081
Human Urine	Detected and Quantified	<5.130				umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	HMDB	21910240
Human Urine	Detected and Quantified	<6				umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	28853722
Human Urine	Detected and Quantified			0	1.8	umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	HMDB	31963255
Human Urine	Detected and Quantified			0	1.9	umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	HMDB	31963255
Human Urine	Detected and Quantified			0	3.1	umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	31963255
Human Urine	Detected and Quantified			0	4.3	umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	31963255
Human Blood	Detected and Quantified	0.14		0.04	0.26	uM	Adult (>18 years old)	Both	Normal	HMDB	8412012
Human Cerebrospinal fluid (csf)	Detected and Quantified			0.15	0.55	uM	Adolescent (13-18 years old)	Female	Normal	HMDB	24613099
Human Blood	Detected and Quantified	0.17		0.073	0.27	uM	Adult (>18 years old)	Both	Normal	HMDB	8345822
Human Urine	Detected and Quantified	0.17	0.11			umol/mmol creatinine	Adolescent (13-18 years old)	Both	Normal	HMDB	16139832
Human Blood	Detected and Quantified	0.187	0.084			uM	Adult (>18 years old)	Both	Normal	HMDB	8448848
Human Blood	Detected and Quantified	0.200		0.00	0.400	uM	Adult (>18 years old)	Not Specified	Normal	HMDB	11067816
Human Cerebrospinal fluid (csf)	Detected and Quantified	0.32	0.11			uM	Adult (>18 years old)	Both	Normal	HMDB	16227558
Human Cerebrospinal fluid (csf)	Detected and Quantified	0.40		0.16	0.59	uM	Adult (>18 years old)	Both	Normal	HMDB	8345822
Human Urine	Detected and Quantified			0.42	19.74	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	32340350
Human Cerebrospinal fluid (csf)	Detected and Quantified	0.51	0.13			uM	Adult (>18 years old)	Both	Normal	HMDB	16227558
Human Urine	Detected and Quantified	0.64	0.39			umol/mmol creatinine	Children (1-13 years old)	Both	Normal	HMDB	16139832
Human Urine	Detected and Quantified	0.92	1.97			umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	2026685
Human Urine	Detected and Quantified	1.28		0.52	6.64	umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	HMDB	32340350
Human Urine	Detected and Quantified	1.3		0.5	2.1	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected and Quantified			1.5	2.4	umol/mmol creatinine	Adult (>18 years old)	Male	Normal	HMDB	27012787
Human Urine	Detected and Quantified	1.6		0	3.6	umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	HMDB	11067816
Human Urine	Detected and Quantified			1.7	2.8	umol/mmol creatinine	Adult (>18 years old)	Female	Normal	HMDB	27012787
Human Urine	Detected and Quantified	1.8		0.00	3.6	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	11067816
Human Urine	Detected and Quantified	1.89		0.42	22.51	umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	HMDB	32340350
Human Urine	Detected and Quantified	1.9		0.7	3.5	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected and Quantified	2.51	1.01			umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	16139832
Human Urine	Detected and Quantified	29.9		0.1	78.9	umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	3.086	1.081			umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	16460720
Human Urine	Detected and Quantified	3.495	2.595			umol/mmol creatinine	Adult (>18 years old)	Both		HMDB	9439441
Human Urine	Detected and Quantified	3.7		1.1	21.6	umol/mmol creatinine	Adolescent (13-18 years old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	8.2		1.5	30.8	umol/mmol creatinine	Children (1-13 years old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	9.4		0.1	79.4	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	10.435	7.593			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	97.67	89.432			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	16.0		1.5	30.8	umol/mmol creatinine	Adult (>18 years old)	Both	Cobalamin malabsorption	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	275.0		50.0	500.0	umol/mmol creatinine	Children (1-13 years old)	Both	Cobalamin malabsorption	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	0.765	0.896			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	9.247	8.216			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	<5.13				umol/mmol creatinine	Children (1 - 18 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Urine	Detected and Quantified	1.58		0.0	3.29	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Urine	Detected and Quantified	2.0	0.7			umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Urine	Detected and Quantified	4.0	0.9			umol/mmol creatinine	Children (1-13 years old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Urine	Detected and Quantified	2.5				umol/mmol creatinine	Adult (>18 years old)	Male	Normal	HMDB	Shaykhutdinov RA, MacInnis GD, Dowlatabadi R, Weljie AM, Vogel HJ. Quantitative analysis of metabolite concentrations in human urine samples using 13C{1H} NMR spectroscopy. Metabolomics. 2009
Human Kidney	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Liver	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
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 Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	21389975
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	9439441
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						Adult (>18 years old)	Both	Normal	HMDB	32966057

External Links

HMDB ID

HMDB0000202

DrugBank ID

DB04183

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB021905

KNApSAcK ID

Not Available

Chemspider ID

473

KEGG Compound ID

C02170

BioCyc ID

CPD-546

BiGG ID

Not Available

Wikipedia Link

Methylmalonic_acid

METLIN ID

3712

PubChem Compound

487

PDB ID

Not Available

ChEBI ID

30860

Food Biomarker Ontology

Not Available

References

Synthesis Reference

Not Available

General References

Leupold D: [Methylmalonic aciduria. Classification, diagnosis and therapy (author's transl)]. Klin Wochenschr. 1977 Jan 15;55(2):57-63. doi: 10.1007/BF01469083. [PubMed:319293 ]
Haurani FI, Hall CA, Rubin R: Megaloblastic anemia as a result of an abnormal transcobalamin II (Cardeza). J Clin Invest. 1979 Nov;64(5):1253-9. doi: 10.1172/JCI109580. [PubMed:500809 ]
Frenkel EP, Kitchens RL: Applicability of an enzymatic quantitation of methylmalonic, propionic, and acetic acids in normal and megaloblastic states. Blood. 1977 Jan;49(1):125-37. [PubMed:830370 ]
Nakamura E, Rosenberg LE, Tanaka K: Microdetermination of methylmalonic acid and other short chain dicarboxylic acids by gas chromatography: use in prenatal diagnosis of methylmalonic acidemia and in studies of isovaleric acidemia. Clin Chim Acta. 1976 Apr 15;68(2):127-40. doi: 10.1016/0009-8981(76)90412-5. [PubMed:1261058 ]
Bennett MJ, Ragni MC, Hood I, Hale DE: Comparison of post-mortem urinary and vitreous humour organic acids. Ann Clin Biochem. 1992 Sep;29 ( Pt 5):541-5. doi: 10.1177/000456329202900509. [PubMed:1444166 ]
Coude MM, Charpentier C, Bonnefont JP, Cheron G, Kamoun P: Organic acids in aqueous humour and plasma: post mortem study in infants and diagnosis of enzymopathies. J Inherit Metab Dis. 1991;14(5):668-73. doi: 10.1007/BF01799931. [PubMed:1779612 ]
Stabler SP, Marcell PD, Podell ER, Allen RH, Lindenbaum J: Assay of methylmalonic acid in the serum of patients with cobalamin deficiency using capillary gas chromatography-mass spectrometry. J Clin Invest. 1986 May;77(5):1606-12. doi: 10.1172/JCI112476. [PubMed:3700655 ]
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Showing metabocard for Methylmalonic acid (MMDBc0000502)

MOL for #<Metabolite:0x00007f9232204ad8>

3D MOL for #<Metabolite:0x00007f9232204ad8>

3D SDF for #<Metabolite:0x00007f9232204ad8>

3D-SDF for #<Metabolite:0x00007f9232204ad8>

PDB for #<Metabolite:0x00007f9232204ad8>

3D PDB for #<Metabolite:0x00007f9232204ad8>

SMILES for #<Metabolite:0x00007f9232204ad8>

INCHI for #<Metabolite:0x00007f9232204ad8>

Structure for #<Metabolite:0x00007f9232204ad8>

3D Structure for #<Metabolite:0x00007f9232204ad8>