CHEBI:30860 - methylmalonic acid

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ChEBI Name methylmalonic acid
ChEBI ID CHEBI:30860
Definition A dicarboxylic acid that is malonic acid in which one of the methylene hydrogens is substituted by a methyl group.
Stars This entity has been manually annotated by the ChEBI Team.
Secondary ChEBI IDs CHEBI:42270, CHEBI:6881, CHEBI:25319
Supplier Information No supplier information found for this compound.
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Lysine (symbol Lys or K) is an α-amino acid that is a precursor to many proteins. Lysine contains an α-amino group (which is in the protonated −NH+3 form when the lysine is dissolved in water at physiological pH), an α-carboxylic acid group (which is in the deprotonated −COO− form when the lysine is dissolved in water at physiological pH), and a side chain (CH2)4NH2 (which is partially protonated when the lysine is dissolved in water at physiological pH), and so it is classified as a basic, charged (in water at physiological pH), aliphatic amino acid. It is encoded by the codons AAA and AAG. Like almost all other amino acids, the α-carbon is chiral and lysine may refer to either enantiomer or a racemic mixture of both. For the purpose of this article, lysine will refer to the biologically active enantiomer L-lysine, where the α-carbon is in the S configuration. The human body cannot synthesize lysine. It is essential in humans and must therefore be obtained from the diet. In organisms that synthesise lysine, two main biosynthetic pathways exist, the diaminopimelate and α-aminoadipate pathways, which employ distinct enzymes and substrates and are found in diverse organisms. Lysine catabolism occurs through one of several pathways, the most common of which is the saccharopine pathway. Lysine plays several roles in humans, most importantly proteinogenesis, but also in the crosslinking of collagen polypeptides, uptake of essential mineral nutrients, and in the production of carnitine, which is key in fatty acid metabolism. Lysine is also often involved in histone modifications, and thus, impacts the epigenome. The ε-amino group often participates in hydrogen bonding and as a general base in catalysis. The ε-ammonium group (−NH+3) is attached to the fourth carbon from the α-carbon, which is attached to the carboxyl (−COOH) group. Due to its importance in several biological processes, a lack of lysine can lead to several disease states including defective connective tissues, impaired fatty acid metabolism, anaemia, and systemic protein-energy deficiency. In contrast, an overabundance of lysine, caused by ineffective catabolism, can cause severe neurological disorders. Lysine was first isolated by the German biological chemist Ferdinand Heinrich Edmund Drechsel in 1889 from hydrolysis of the protein casein, and thus named it Lysin, from Greek λύσις (lysis) 'loosening'. In 1902, the German chemists Emil Fischer and Fritz Weigert determined lysine's chemical structure by synthesizing it. The one-letter symbol K was assigned to lysine for being alphabetically nearest, with L being assigned to the structurally simpler leucine, and M to methionine.
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Formula C4H6O4
Net Charge 0
Average Mass 118.08804
Monoisotopic Mass 118.02661
InChI InChI=1S/C4H6O4/c1-2(3(5)6)4(7)8/h2H,1H3,(H,5,6)(H,7,8)
InChIKey ZIYVHBGGAOATLY-UHFFFAOYSA-N
SMILES CC(C(O)=O)C(O)=O
Metabolite of Species Details
Homo sapiens (NCBI:txid9606) See: DOI
Roles Classification
Chemical Role(s): Bronsted acid
A molecular entity capable of donating a hydron to an acceptor (Bronsted base).
(via oxoacid )
Biological Role(s): human metabolite
Any mammalian metabolite produced during a metabolic reaction in humans (Homo sapiens).
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ChEBI Ontology
Outgoing methylmalonic acid (CHEBI:30860) has functional parent malonic acid (CHEBI:30794)
methylmalonic acid (CHEBI:30860) has role human metabolite (CHEBI:77746)
methylmalonic acid (CHEBI:30860) is a C4-dicarboxylic acid (CHEBI:66873)
methylmalonic acid (CHEBI:30860) is conjugate acid of methylmalonate(1−) (CHEBI:30861)
Incoming O-methylmalonyl-L-carnitine (CHEBI:85473) has functional parent methylmalonic acid (CHEBI:30860)
aryl(methyl)malonic acid (CHEBI:15849) has functional parent methylmalonic acid (CHEBI:30860)
methylmalonyl-CoA (CHEBI:16625) has functional parent methylmalonic acid (CHEBI:30860)
methylmalonate(1−) (CHEBI:30861) is conjugate base of methylmalonic acid (CHEBI:30860)
IUPAC Name
methylpropanedioic acid
Synonyms Sources
1,1-Ethanedicarboxylic acid HMDB
2-methylmalonic acid NIST Chemistry WebBook
α-methylmalonic acid ChEBI
Isosuccinic acid HMDB
Methylmalonic acid KEGG COMPOUND
METHYLMALONIC ACID PDBeChem
Manual Xrefs Databases
C02170 KEGG COMPOUND
CPD-546 MetaCyc
DB04183 DrugBank
DXX PDBeChem
HMDB0000202 HMDB
LMFA01170118 LIPID MAPS
Methylmalonic_acid Wikipedia
View more database links
Registry Numbers Types Sources
1756084 Reaxys Registry Number Reaxys
50008 Gmelin Registry Number Gmelin
516-05-2 CAS Registry Number KEGG COMPOUND
516-05-2 CAS Registry Number NIST Chemistry WebBook
516-05-2 CAS Registry Number ChemIDplus
Citations Types Sources
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Last Modified
23 October 2015