Disorders of mitochondrial oxidative phosphorylation

Definition

An inborn error of metabolism in cellular respiration (oxidative phosphorylation) in the mitochondria, where a series of enzymes catalyze the transfer of electrons to molecular oxygen and the generation of energy-storing ATP

8 subordinate codes

• 5C53.20Mitochondrial DNA depletion syndromes
• 5C53.21Multiple mitochondrial DNA deletion syndromes
• 5C53.22Coenzyme Q10 deficiency
• 5C53.23Mitochondrial protein translation defects
• 5C53.24Leigh syndrome
• 5C53.25Isolated ATP synthase deficiency
• 4B4YOther specified diseases of the immune system
• 4B4ZDiseases of the immune system, unspecified

Nearby in Inborn errors of energy metabolism

• 5C53.0Disorders of pyruvate metabolism
• 5C53.1Disorders of the citric acid cycle
• 5C53.3Disorders of mitochondrial membrane transport
• 5C53.4Disorders of creatine metabolism
• 4B4YOther specified diseases of the immune system
• 4B4ZDiseases of the immune system, unspecified