Disorders of mitochondrial fatty-acid oxidation

Definition:

Disorders of mitochondrial fatty-acid oxidation are a group of rare inherited conditions that lead to accumulation of fatty acids and decreases in cell energy metabolism due to enzyme or transporter defects in the fatty acid metabolic pathway. The symptoms and treatment vary between different fatty acid oxidation disorders.

References:

[1]. Allan Meldgaard Lund, et al. Clinical and biochemical monitoring of patients with fatty acid oxidation disorders. J Inherit Metab Dis. 2010 Oct;33(5):495-500. [Content Brief]

[2]. Bridget Wilcken, et al. Fatty acid oxidation disorders: outcome and long-term prognosis. J Inherit Metab Dis. 2010 Oct;33(5):501-6. [Content Brief]

[3]. Michelle Kompare, et al. Mitochondrial fatty-acid oxidation disorders. Semin Pediatr Neurol. 2008 Sep;15(3):140-9. [Content Brief]

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