DLX2 belongs to the six-member family of DLX genes characterized by a homeobox related to that found in the insect Distal-less (Dll) gene. It was the first human homologue to be discovered (Selski et al., 1993; McGuinness et al., 1996).The six DLX genes are organized as three bigenic pairs with a tail-to-tail orientation (Zerucha et al., 2000) and located on chromosomes where HOX clusters are also found (DLX5/DLX6; 7q21.3, syntenic to the HOXA cluster), (DLX1/DLX2; 2q32 syntenic to the HOXD cluster; Simeone et al., 1994; Zerucha et al., 2000) and (DLX3/DLX4; 17q21.33 syntenic to the HOXB cluster).During embryonic development DLX genes are involved in the control of appendage and craniofacial morphogenesis and in the differentiation of reproductive organs; in the adult they play a role in bone homeostasis and in the maintenance of tissue.