Methylmalonic acid

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Methylmalonic acid
Names
Preferred IUPAC name
Methylpropanedioic acid
Other names
Methylmalonic acid
Identifiers
3D model (
JSmol
)
ChEBI
ChemSpider
ECHA InfoCard
 100.007.473 Edit this at Wikidata
EC Number
  • 208-219-5
KEGG
MeSH Methylmalonic+acid
UNII
  • InChI=1S/C4H6O4/c1-2(3(5)6)4(7)8/h2H,1H3,(H,5,6)(H,7,8) ☒N
    Key: ZIYVHBGGAOATLY-UHFFFAOYSA-N ☒N
  • InChI=1/C4H6O4/c1-2(3(5)6)4(7)8/h2H,1H3,(H,5,6)(H,7,8)
    Key: ZIYVHBGGAOATLY-UHFFFAOYAT
  • CC(C(=O)O)C(=O)O
Properties
C4H6O4
Molar mass 118.088 g/mol
Density 1.455 g/cm−3
Melting point 134 °C (273 °F; 407 K)
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 checkY☒N ?)

Methylmalonic acid (MMA) (

conjugate base methylmalonate) is a dicarboxylic acid that is a C-methylated derivative of malonic acid
.

Metabolism

Methylmalonic acid pathway

Methylmalonic acid is a by-product of certain metabolic processes. The sources of this include the following:

Krebs cycle, and is thus part of one of the anaplerotic reactions. In addition, the enzyme ACSF3 converts methylmalonic acid back into methymalonyl-CoA.[3]

Intracellular esterases are capable to remove the methyl group from methylmalonic acid and thus generate malonic acid.[4]

Clinical relevance

Vitamin B12 deficiency

Increased methylmalonic acid levels may indicate a vitamin B12 deficiency. However, it is sensitive (those with the deficiency almost always test positive) but not specific (those that do not have vitamin B12 deficiency may have elevated levels of methylmalonic acid detected).[5] 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.[6]

Metabolic diseases

An excess is associated with

methylmalonic acidemia
.

If elevated methylmalonic acid levels are accompanied by elevated

methylmalonic acidemia.[7]

Cancer

Moreover, MMA accumulation in the blood with age has been linked with tumour progression in 2020.[8]

Bacterial overgrowth in the small intestine

propionate, which is a precursor to methylmalonic acid.[9][10] It has been shown that in these cases, methylmalonic acid levels returned to normal with the administration of metronidazole.[9][11]

Measurement

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 and 271 nmol/L.[12][13]

See also

References

  1. ^ a b "Dissociation Constants Of Organic Acids And Bases". ZirChrom Separations, Inc.
  2. ^
    PMID 25205257
    .
  3. ^ "ACSF3 gene". Medlineplus. Retrieved 2024-04-15.
  4. PMID 9692761
    .
  5. ^ "Sensitivity and Specificity". Emory University School of Medicine. Archived from the original on 1 October 2012.
  6. ^ "B12 Deficiency and Dizziness". www.dizziness-and-balance.com.
  7. PMID 26915364
    .
  8. PMID 32814897
    .
  9. ^
    PMID 19322060
    .
  10. PMID 4629318
    .
  11. PMID 29129351
    .
  12. doi:10.22028/D291-20838
    .
  13. ^ "Methylmalonic Acid, Serum or Plasma (Vitamin B12 Status)". ltd.aruplab.com.

Further reading

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