Dental enamel is a highly mineralized tissue with most of its volume occupied by large, highly organized, hydroxyapatite crystals. This structure is thought to be controlled through the interaction of many organic matrix molecules including amelogenin, ameloblastin, enamelin, tuftelin and several other enzymes. All of these secreted proteins are involved in the mineralization and enamel matrix formation in developing tooth enamel. Ameloblastin (AMBN), which localizes to the extracellular matrix, is an ameloblast-specific protein. It is detected in the sheath space between rod-interrod enamel and at the Tomes processes of secretory ameloblasts. Defects in the gene encoding for ameloblastin, AMBN, can be seen in patients with ameloblastomas.