Acylcarnitine Structure, Blood


Carnitine is critical for normal skeletal muscle bioenergetics. Carnitine has a dual role as it is required for long-chain fatty acid oxidation, and shuttles accumulated acyl groups out of the mitochondria. Muscle requires optimisation of both of these metabolic processes during peak exercise performance. All biochemical actions of carnitine are based on the carnitine acyltransferase – meditated reversible transfer of carboxylic acids, or “acyl” groups, between carnitine and coenzyme A. Thus, the carnitine pool consists of carnitine, short-chain acylcarnitines, and longchain acylcarnitines.


Elevations in specific acylcarnitines can be diagnostic for several fatty acid oxidation disorders and organic acidurias. Measurement of acylcarnitines may be helpful in the evaluation of patients with hypoglycaemia, cardiomyopathy, myopathy, rhabdomyolysis (breakdown of muscle fibre), and infants with ketonuria, hyperammonemia, or acidosis.

Sample Type, Quantity & Conditions

1 ml EDTA Plasma Frozen

Special Precautions

Freeze immediately

Normal Range

By Report

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