Teneurins are a novel family of transmembrane proteins expressed during pattern formation and morphogenesis. Originally discovered as ten-m and ten-a in Drosophila, four vertebrate teneurins as well as a Caenorhabditis elegans homologue were identified. The conserved domain architecture of teneurins includes an intracellular domain containing polyproline motifs. The long extracellular domain consists of eight EGF-like repeats, a region of conserved cysteines and unique YD-repeats. Vertebrate teneurins are most prominently expressed in the developing central nervous system, but are also expressed in developing limbs. In C. elegans, RNAi experiments and studies of mutants reveal that teneurins are required during fundamental developmental processes like cell migration and axon pathfinding. Cell culture experiments suggest that the intracellular domain of teneurins translocates to the nucleus following release from the membrane by proteolytic processing. Interestingly, the human teneurin-1 gene is located on the X-chromosome in a region where several families with X-linked mental retardation are mapped.