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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:02:20 UTC
HMDB IDHMDB00227
Secondary Accession NumbersNone
Metabolite Identification
Common NameMevalonic acid
DescriptionMevalonic acid is a key organic compound in biochemistry. It is a precursor in the biosynthetic pathway, known as the HMG-CoA reductase pathway, that produces terpenes and steroids. Mevalonate is produced by NADPH from 3-hydroxy-3-methylglutaryl CoA via reduction. This reaction occurs in the cytosol. It is the committed step in cholesterol synthesis, -- Wikipedia The production of mevalonic acid (MVA) by the enzyme 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) reductase, is the rate-limiting step in the biosynthesis of cholesterol. (Jemal et. al, Rapid Communications in Mass Spectrometry, 2003, 17:1715) Plasma concentrations and urinary excretion of MVA are decreased by HMG-CoA reductase inhibitor drugs such as pravastatin, simvastatin and atorvastatin. Naoumova RP, Marais AD, Mountney J, Firth JC, Rendell NB, Taylor GW, Thompson GR. Atherosclerosis 1996; 119: 203.
Structure
Thumb
Synonyms
ValueSource
DL-Mevalonic acidChEBI
MVAChEBI
RS-Mevalonic acidChEBI
DL-MevalonateGenerator
3,5-Dihydroxy-3-methylpentanoateGenerator
RS-MevalonateGenerator
(3Rs)-MevalonateHMDB
(3Rs)-Mevalonic acidHMDB
2,4-Dideoxy-3-C-methylpentonateHMDB
2,4-Dideoxy-3-C-methylpentonic acidHMDB
3,5-Dihydroxy-3-methyl-valerateHMDB
3,5-Dihydroxy-3-methyl-valeric acidHMDB
3,5-Dihydroxy-3-methylvalerateHMDB
3,5-Dihydroxy-3-methylvaleric acidHMDB
b,D-Dihydroxy-b-methylvalerateHMDB
b,D-Dihydroxy-b-methylvaleric acidHMDB
b,D-Dihydroxy-beta-methylvalerateHMDB
b,D-Dihydroxy-beta-methylvaleric acidHMDB
Hiochic acidHMDB
MevalonateHMDB
MK 91HMDB
MVSHMDB
Chemical FormulaC6H12O4
Average Molecular Weight148.1571
Monoisotopic Molecular Weight148.073558872
IUPAC Name3,5-dihydroxy-3-methylpentanoic acid
Traditional NameDL-mevalonic acid
CAS Registry Number150-97-0
SMILES
CC(O)(CCO)CC(O)=O
InChI Identifier
InChI=1S/C6H12O4/c1-6(10,2-3-7)4-5(8)9/h7,10H,2-4H2,1H3,(H,8,9)
InChI KeyInChIKey=KJTLQQUUPVSXIM-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as hydroxy fatty acids. These are fatty acids in which the chain bears a hydroxyl group.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acids and conjugates
Direct ParentHydroxy fatty acids
Alternative Parents
Substituents
  • Hydroxy fatty acid
  • Methyl-branched fatty acid
  • Short-chain hydroxy acid
  • Branched fatty acid
  • Beta-hydroxy acid
  • Hydroxy acid
  • Tertiary alcohol
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Hydrocarbon derivative
  • Primary alcohol
  • Organooxygen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
Biofunction
  • Steroid biosynthesis
ApplicationNot Available
Cellular locations
  • Endoplasmic reticulum
  • Peroxisome
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point24 - 27 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility414.0 mg/mLALOGPS
logP-0.9ALOGPS
logP-1.1ChemAxon
logS0.45ALOGPS
pKa (Strongest Acidic)4.38ChemAxon
pKa (Strongest Basic)-2.4ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area77.76 Å2ChemAxon
Rotatable Bond Count4ChemAxon
Refractivity34.51 m3·mol-1ChemAxon
Polarizability14.54 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0002-2900000000-d1dc97020c103f2a916bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-014i-9000000000-b4dc45e964aee6759d93View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-014i-9100000000-98f7e8ee013680f4f610View in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Endoplasmic reticulum
  • Peroxisome
Biofluid Locations
  • Blood
  • Urine
Tissue Location
  • Fibroblasts
  • Liver
  • Lymphocyte
Pathways
NameSMPDB LinkKEGG Link
Alendronate pathwaySMP00095Not Available
Atorvastatin PathwaySMP00131Not Available
Cerivastatin PathwaySMP00111Not Available
CHILD SyndromeSMP00387Not Available
Cholesteryl ester storage diseaseSMP00508Not Available
Chondrodysplasia Punctata II, X Linked Dominant (CDPX2)SMP00388Not Available
DesmosterolosisSMP00386Not Available
Fluvastatin PathwaySMP00119Not Available
Hyper-IgD syndromeSMP00509Not Available
HypercholesterolemiaSMP00209Not Available
Ibandronate PathwaySMP00079Not Available
Lovastatin PathwaySMP00099Not Available
Lysosomal Acid Lipase Deficiency (Wolman Disease)SMP00319Not Available
Mevalonic aciduriaSMP00510Not Available
Pamidronate PathwaySMP00117Not Available
Pravastatin PathwaySMP00089Not Available
Risedronate PathwaySMP00112Not Available
Rosuvastatin PathwaySMP00092Not Available
Simvastatin Action PathwaySMP00082Not Available
Smith-Lemli-Opitz Syndrome (SLOS)SMP00389Not Available
Steroid BiosynthesisSMP00023map00100
Wolman diseaseSMP00511Not Available
Zoledronate PathwaySMP00107Not Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.028 +/- 0.006 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.043 +/- 0.013 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified0.14 (0.06-0.22) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.5 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.0531 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.178 (0.0515-0.545) umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.012 +/- 0.002 uMAdult (>18 years old)BothSmith-Lemli-Opitz syndrome details
Associated Disorders and Diseases
Disease References
Smith-Lemli-Opitz syndrome
  1. Honda M, Tint GS, Honda A, Salen G, Shefer S, Batta AK, Matsuzaki Y, Tanaka N: Regulation of cholesterol biosynthetic pathway in patients with the Smith-Lemli-Opitz syndrome. J Inherit Metab Dis. 2000 Jul;23(5):464-74. [10947201 ]
Associated OMIM IDs
  • 270400 (Smith-Lemli-Opitz syndrome)
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB005126
KNApSAcK IDNot Available
Chemspider ID436
KEGG Compound IDC00418
BioCyc IDMEVALONATE
BiGG ID34923
Wikipedia LinkMevalonic acid
NuGOwiki LinkHMDB00227
Metagene LinkHMDB00227
METLIN ID127
PubChem Compound449
PDB IDNot Available
ChEBI ID25351
References
Synthesis ReferenceTakahara, Kenji; Nakamura, Yoshio; Ohashi, Takehisa; Watanabe, Kiyoshi. Fermentative production of mevalonic acid. Jpn. Kokai Tokkyo Koho (1985), 4 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Houten SM, Kuis W, Duran M, de Koning TJ, van Royen-Kerkhof A, Romeijn GJ, Frenkel J, Dorland L, de Barse MM, Huijbers WA, Rijkers GT, Waterham HR, Wanders RJ, Poll-The BT: Mutations in MVK, encoding mevalonate kinase, cause hyperimmunoglobulinaemia D and periodic fever syndrome. Nat Genet. 1999 Jun;22(2):175-7. [10369261 ]
  2. Hoffmann G, Gibson KM, Brandt IK, Bader PI, Wappner RS, Sweetman L: Mevalonic aciduria--an inborn error of cholesterol and nonsterol isoprene biosynthesis. N Engl J Med. 1986 Jun 19;314(25):1610-4. [3012338 ]
  3. Dmitrieva NA, Perrun'kina AM, Khomulo PS: [Determination of mevalonic acid in the urine and its concentration in the urine of patients with atherosclerosis] Vopr Med Khim. 1968 Jan-Feb;14(1):106-8. [5683362 ]
  4. Larson RA, Chung J, Scanu AM, Yachnin S: Neutrophils are required for the DNA synthetic response of human lymphocytes to mevalonic acid: evidence suggesting that a nonsterol product of mevalonate is involved. Proc Natl Acad Sci U S A. 1982 May;79(9):3028-32. [6953445 ]
  5. Siavoshian S, Simoneau C, Maugeais P, Marks L, Rodary L, Gardette J, Krempf M: Measurement of mevalonic acid in human urine by bench top gas chromatography-mass spectrometry. Clin Chim Acta. 1995 Dec 29;243(2):129-36. [8747489 ]
  6. Mitchell ED Jr, Avigan J: Control of phosphorylation and decarboxylation of mevalonic acid and its metabolites in cultured human fibroblasts and in rat liver in vivo. J Biol Chem. 1981 Jun 25;256(12):6170-3. [6263908 ]
  7. Abrar M, Martin PD: Validation and application of an assay for the determination of mevalonic acid in human plasma by liquid chromatography tandem mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jun 25;773(2):103-11. [12031835 ]
  8. Haas D, Kelley RI, Hoffmann GF: Inherited disorders of cholesterol biosynthesis. Neuropediatrics. 2001 Jun;32(3):113-22. [11521206 ]
  9. Larson RA, Kluskens LE, Yachnin S: The DNA synthetic response of normal and abnormal human lymphocytes to mevalonic acid: the role of granulocytes as a helper population. J Allergy Clin Immunol. 1984 Sep;74(3 Pt 1):280-90. [6236250 ]
  10. Larson RA, Yachnin S: Mevalonic acid induces DNA synthesis in chronic lymphocytic leukemia cells. Blood. 1984 Jul;64(1):257-62. [6610447 ]
  11. Parker TS, McNamara DJ, Brown CD, Kolb R, Ahrens EH Jr, Alberts AW, Tobert J, Chen J, De Schepper PJ: Plasma mevalonate as a measure of cholesterol synthesis in man. J Clin Invest. 1984 Sep;74(3):795-804. [6565710 ]
  12. Hoffmann GF, Sweetman L, Bremer HJ, Hunneman DH, Hyanek J, Kozich V, Lehnert W, Nyhan WL, Speidel I, Trefz FK, et al.: Facts and artefacts in mevalonic aciduria: development of a stable isotope dilution GCMS assay for mevalonic acid and its application to physiological fluids, tissue samples, prenatal diagnosis and carrier detection. Clin Chim Acta. 1991 May 15;198(3):209-27. [1653652 ]
  13. Jemal M, Schuster A, Whigan DB: Liquid chromatography/tandem mass spectrometry methods for quantitation of mevalonic acid in human plasma and urine: method validation, demonstration of using a surrogate analyte, and demonstration of unacceptable matrix effect in spite of use of a stable isotope analog internal standard. Rapid Commun Mass Spectrom. 2003;17(15):1723-34. [12872277 ]
  14. Lindenthal B, von Bergmann K: Urinary excretion and serum concentration of mevalonic acid during acute intake of alcohol. Metabolism. 2000 Jan;49(1):62-6. [10647065 ]

Enzymes

General function:
Involved in hydroxymethylglutaryl-CoA reductase (NADPH) activity
Specific function:
Transmembrane glycoprotein that is the rate-limiting enzyme in cholesterol biosynthesis as well as in the biosynthesis of nonsterol isoprenoids that are essential for normal cell function including ubiquinone and geranylgeranyl proteins.
Gene Name:
HMGCR
Uniprot ID:
P04035
Molecular weight:
97475.155
General function:
Involved in ATP binding
Specific function:
May be a regulatory site in cholesterol biosynthetic pathway.
Gene Name:
MVK
Uniprot ID:
Q03426
Molecular weight:
42450.475