Under normal circumstances, the biological function of ANT1 is to form nonspecific pores that cause mitochondrial inner membrane permeability, output ATP to exchange for cytoplasmic ADP, and act as an electric pump.440ANT1 mutations cause mitochondrial myopathy (MM), characterized by ragged red muscular fibers and progressive external ophthalmoplegia due to extraocular muscle paralysis. The gene discussed is PRPF6; the disease is Miyoshi myopathy.