Table 1

Distribution of genetic lesions in components of the respiratory chain

Causes of Leigh syndrome
DefectTransmissionFrequency
Complex IAR, M++++
Complex IIAR+
Complex IVAR+++
Complex VM, AR++
tRNALeu(UUR)M+
tRNALysM+
CoQ10AR+
PDHCXR, AR+++

  • In Leigh's syndrome. Inheritance: AR, autosomal recessive; CoQ10, coenzyme Q10; M, maternal; PDHC, pyruvate dehydrogenase complex; XR, X-liked recessive.