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.
Category
Inherited metabolic disorder
Brite
Human diseases in ICD-11 classification [BR:br08403]
05 Endocrine, nutritional or metabolic diseases
Metabolic disorders
Inborn errors of metabolism
5C52 Inborn errors of lipid metabolism
H00525 Disorders of mitochondrial fatty-acid oxidation
Pathway-based classification of diseases [BR:br08402]
Lipid/glycolipid metabolism
nt06020 beta-Oxidation in mitochondria
H00525 Disorders of mitochondrial fatty-acid oxidation