AGK and Congenital cataract - hypertrophic cardiomyopathy - mitochondrial myopathy: Since the TIM22 complex is a multisubunit molecular machine specialized for the translocation of proteins with internal targeting signals into the inner mitochondrial membrane, defects in AGK can result in defective mitochondrial protein import and the possibility of developing Sengers syndrome due to depletion of TIM22 complex substrates.