Ethylmalonic acid (EMA) is an organic acid that appears in urine when certain metabolic pathways are disrupted, particularly those involving fatty acid oxidation and mitochondrial energy production. Under normal conditions, the body efficiently breaks down fatty acids to produce energy. However, when this process is impaired, intermediate byproducts—including ethylmalonic acid—can build up and spill into urine.
In clinical practice, elevated EMA is considered a marker of mitochondrial stress, impaired fat metabolism, or nutrient deficiencies that affect energy production.
The Organic Acids Test (OAT) is designed to evaluate how well the body is producing and utilizing energy at a cellular level. EMA is included because it is a sensitive indicator of:
Fatty acid metabolism – how well the body is breaking down fats for fuel.
Mitochondrial function – since mitochondria are responsible for converting fatty acids into usable energy (ATP).
Nutrient status – certain vitamins and cofactors are needed for proper fat oxidation; deficiencies may elevate EMA.
Possible inborn errors of metabolism – in rare cases, very high EMA may signal genetic conditions affecting energy metabolism.
By analyzing EMA along with other organic acids, clinicians can better understand whether symptoms like fatigue, weakness, or developmental issues may be related to underlying energy deficits.
Nutrient deficiencies – particularly riboflavin (B2) and carnitine
Mitochondrial health – genetic or acquired dysfunction
Dietary intake – very high-fat diets may stress fat oxidation pathways in vulnerable individuals
Oxidative stress – increases metabolic strain on mitochondria
Age – mitochondrial efficiency naturally declines with aging
If EMA is elevated, supportive strategies may include:
Riboflavin supplementation – correcting vitamin B2 deficiency can normalize fatty acid metabolism.
Carnitine support – oral carnitine may help improve fatty acid transport into mitochondria.
Mitochondrial nutrients – coenzyme Q10, alpha-lipoic acid, and niacin may enhance energy production.
Balanced diet – ensuring adequate but not excessive dietary fat and supporting overall macronutrient balance.
Reducing oxidative stress – antioxidants and lifestyle interventions (exercise, sleep, stress management) can protect mitochondria.
Because EMA abnormalities may reflect deeper metabolic issues, especially when significantly elevated, results should be interpreted by a healthcare provider in the context of symptoms and other organic acid markers.
Ethylmalonic acid is a key mitochondrial biomarker on the Organic Acids Test (OAT). Elevated levels point to impaired fatty acid metabolism, often related to nutrient deficiencies (riboflavin, carnitine), mitochondrial dysfunction, or—in rare cases—genetic metabolic conditions. Identifying and addressing these issues can improve energy levels, muscle function, and overall cellular health.
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Elevated urinary EMA is most commonly associated with impaired fatty acid oxidation. This means the body struggles to efficiently convert fats into energy, forcing metabolic detours that produce ethylmalonic acid.
Possible causes of high EMA include:
Mitochondrial dysfunction – when cells cannot efficiently produce ATP from fatty acids.
Riboflavin (Vitamin B2) deficiency – riboflavin is a key cofactor in fatty acid oxidation pathways.
Carnitine deficiency or insufficiency – carnitine helps shuttle fatty acids into mitochondria; low levels may cause incomplete fat breakdown.
Other nutrient imbalances – such as coenzyme Q10 or niacin, which support mitochondrial energy production.
Genetic metabolic conditions – rare inherited disorders like multiple acyl-CoA dehydrogenase deficiency (MADD) or ethylmalonic encephalopathy.
Clinical associations of elevated EMA may include:
Chronic fatigue or low energy
Exercise intolerance
Muscle weakness or pain
Developmental delays in children (when very elevated)
Neurological symptoms in severe metabolic conditions
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Low or normal EMA is usually considered non-pathological and simply indicates that fatty acid metabolism is functioning efficiently. Unlike elevated results, low values do not generally carry clinical significance.
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I have been using Healthmatters.io since 2021. I travel all over the world and use different doctors and health facilities. This site has allowed me to consolidate all my various test results over 14 years in one place. And every doctor that I show this to has been impressed. Because with any health professional I talk to, I can pull up historical results in seconds. It is invaluable. Even going back to the same doctor, they usually do not have the historical results from their facility in a graph format. That has been very helpful.
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Advanced Plan Member since 2020
What fantastic service and great, easy-to-follow layouts! I love your website; it makes it so helpful to see patterns in my health data. It's truly a pleasure to use. I only wish the NHS was as organized and quick as Healthmatters.io. You've set a new standard for health tracking!
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Healthmatters Pro Member since 2024
As a PRO member and medical practitioner, Healthmatters.io has been an invaluable tool for tracking my clients' data. The layout is intuitive, making it easy to monitor trends and spot patterns over time. The ability to customize reports and charts helps me present information clearly to my clients, improving communication and outcomes. It's streamlined my workflow, saving me time and providing insights at a glance. Highly recommended for any practitioner looking for a comprehensive and user-friendly solution to track patient labs!
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