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Research Ethylmalonic Acid
OMX Organic Metabolomics / Diagnostic Solutions Diagnostic Solutions Laboratory

Ethylmalonic Acid

Optimal Result: 9.9 - 65.6 nmol/mg Creatinine.

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

Ethylmalonate, which comes from the breakdown of butyrate, has a carnitine-dependent pathway and can accumulate with an insufficient amount of carnitine. Dietary fat, carbohydrate, and protein are all broken down to produce energy using pathways that require vitamin B2 (riboflavin).

If you do not have sufficient riboflavin, compounds such as adipate, suberate, and ethylmalonate may increase in urine.

What is Carnitine?

Carnitine helps your body use fatty acids. The body makes small amounts of carnitine.

However, if minimum requirements are not met, carnitine dependent functions fail to proceed normally. Long-chain fatty acids go through beta-oxidation in the mitochondria, which is a carnitine dependent step.

If there are inadequate amounts of carnitine, the long-chain fatty acids will get processed outside of the mitochondria; Adipate and suberate are by-products of long-chain fatty acid breakdown outside of the mitochondria.

Note: These tests are used to check for rare metabolic disorders, usually in infants. There is no apparent reason nor benefit to checking ethylmalonic and methylsuccinic acid levels in adults who aren’t suspected to have rare genetic disorders.

What does it mean if your Ethylmalonic Acid result is too high?

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.

Note: These tests are used to check for rare metabolic disorders, usually in infants. There is no apparent reason nor benefit to checking ethylmalonic and methylsuccinic acid levels in adults who aren’t suspected to have rare genetic disorders.

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