Methylmalonic acid

Methylmalonic acid
Names
IUPAC name
2-Methylpropanedioic acid
Other names
Methylmalonic acid
Identifiers
516-05-2 YesY
ChEBI CHEBI:30860 N
ChemSpider 473 N
EC Number 208-219-5
Jmol interactive 3D Image
KEGG C02170 N
MeSH Methylmalonic+acid
PubChem 487
Properties
C4H6O4
Molar mass 118.088 g/mol
Acidity (pKa) pKa1 = 3,07[1]
pKa2 = 5,76[1]
Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa).
N verify (what is YesYN ?)
Infobox references

Methylmalonic acid (MMA) is a dicarboxylic acid that is a C-methylated derivative of malonate.

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, it enters the Krebs cycle, and is thus part of one of the anaplerotic reactions.

Pathology

Increased methylmalonic acid levels may indicate a vitamin B12 deficiency. However, it is sensitive (those with the disease almost always test positive) but not specific (those that test positive do not always have the disease). MMA is elevated in 90-98% of patients with B12 deficiency. It has lower specificity as 20-25% of patients over the age of 70 have elevated levels of MMA, but 25-33% of them do not have B12 deficiency. For this reason, MMA test is not routinely recommended in the elderly.[2]

An excess is associated with methylmalonic acidemia.

MMA concentrations in blood are measured by Gas chromatographic Mass spectrometry or LC-MS and the expected values of MMA in healthy people are between 73-271 nmol/L. [3][4]

See also

References

  1. 1 2 Dissociation Constants Of Organic Acids and Bases (600 compounds)
  2. ↑ B12 Deficiiency and Dizziness
  3. ↑ http://scidok.sulb.uni-saarland.de/volltexte/2007/1076/pdf/Thesis.pdf
  4. ↑ http://ltd.aruplab.com/Tests/Pub/0099431
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