However, both Sengers syndrome patient fibroblasts harbouring AGK variants and HEK293 AGK knockout cells, where the 440 kDa TIM22 dissociates into a smaller undefined complex, failed to show significantly reduced import and assembly or decreased levels of TIM23 (10). This evidence concerns the gene AGK and Congenital cataract - hypertrophic cardiomyopathy - mitochondrial myopathy.