Pitx2 is an upstream activator of extraocular myogenesis and survival

► Pitx2 prevents apoptosis of extraocular muscle primordia prior to specification. ► Pitx2 activates Muscle Regulatory Factors in the extraocular muscles. ► Both Pitx2 and MRF expression are required to prevent precursor apoptosis. ► Pitx2 is the first identified upstream activator of extraocular myogenesis.