In Senger’s syndrome, an autosomal recessive disease characterized by hypertrophic cardiomyopathy, mitochondrial myopathy, lactic acidosis, and congenital cataracts [111], it appears likely that ANT1 depletion is secondary to a mutation in a gene that functions in processing the translocase. Here, PRPF6 is linked to Congenital cataract - hypertrophic cardiomyopathy - mitochondrial myopathy.