methylmalonic acid


methylmalonic acid

 [meth″il-mah-lon´ik] a carboxylic acid that is a structural isomer of succinic acid; occurring in excess in the blood and other body fluids in methylmalonicacidemia.

meth·yl·ma·lon·ic ac·id

(meth'il-mă-lon'ik as'id), 2-Methylpropanedioic acid, an important intermediate in fatty acid metabolism; seen in elevated levels in cases of vitamin B12 deficiency. Note that methylmalonate is not methyl malonate, which is the dimethyl ester of malonate. Synonym(s): isosuccinic acid

methylmalonic acid

A dicarboxylic acid intermediate in fatty acid metabolism, and a C-methylated derivative of malonate, which is increased in vitamin B12 and/or folate deficiency.
Ref range
• Serum, MA, 80–300 nmol/L; homocysteine, 8–20 µmol/L.
• Urine, MA < 3.6 mmol/mol creatinine.
Specimen
Serum, random urine.
 
Method
GC/MS, HPLC

methylmalonic acid

Isosuccinic acid An intermediate in fatty acid metabolism, which is ↑ in vitamin B12 and/or folate deficiency