Ethylmalonic acid

Adipate, Suberate, and Ethylmalonate elevations indicate metabolic blocks. Carnitine is needed to move fatty acids into the mitochondria where they are converted to energy using vitamin B2. When insufficient levels of carnitine or vitamin B2 slow down this process, other parts of the cellular machinery take over and make adipate and suberate. A similar block in another pathway causes high ethylmalonate. Since most of your body’s energy is produced from the burning of fatty acids, your muscles and brain suffer when this cellular energy pathway is blocked.

Value may be elevated in:

- ketosis, 

- fasting, 

- deficiency of the fat-transporting molecule carnitine, 

- genetic deficiencies of fatty acid metabolism

- the genetic disease multiple acyl dehydrogenase deficiency, 

- excessive intake of adipic acid-containing foods such as Jell-O,

- by increased intake of foods containing medium chain triglycerides such as coconut oil.

Symptoms:

Symptoms include hypoglycemia and lethargy. 

Potential treatment options:

- Supplementation of carnitine and vitamin B2 may be needed when these compounds are too high. Insufficiency of vitamin B2 is implicated in impaired carbohydrate metabolism, migraines, and dementia.

- Carnitine supplementation has been documented to improve Alzheimer’s, age-related cognitive decline, and cardiac function.

Decreased urinary ethylmalonate levels can happen due to:

- Very low-fat diets

- Fat malabsorption

- Defect in isoleucine metabolism

Low levels of ethylmalonate may occur if there are low levels of precursors (adipate, suberate, isoleucine), if there are nutritional enzyme inhibitions, or if a low-activity enzyme variant is inherited. Ethylmalonate, like methylsuccinate, is primarily derived from branched-chain fatty acids (beef, dairy, miso, sauerkraut). Patients on a low-fat diet or with fat malabsorption may have lower ethylmalonate levels. Ethylmalonate is the precursor for methylsuccinate, which is taken up by pancreatic beta-cells.

→ Consider supporting synthesis with vitamins B2, B3, biotin, magnesium and L-carnitine.

Ethylmalonate, together with Adipate and Suberate, gives information about your ability to process fatty acids.