“…Modeling of ADRP mutations in transgenic animals has unequivocally confirmed the crucial role of this signal in the delivery of rhodopsin to the ROS. Transgenic mice, frogs, rats and pigs carrying mutations in the VxPx domain all develop retinal degeneration that is preceded by mislocalization of rhodopsin to multiple cellular compartments, including the photoreceptor synapse, which is normally completely devoid of rhodopsin (Concepcion & Chen, 2010; Concepcion et al, 2002; Green et al, 2000; Lee & Flannery, 2007; Li et al, 1996; Li et al, 1998; Ng et al, 2008; Sommer et al, 2011; Tam et al, 2000). The presence of mislocalized rhodopsin likely causes retinal degeneration in transgenic rods through the impairment of normal cellular functions that depend on the functional compartmentalization of photoreceptor cells.…”