Amelogenin (AMEL) is the most abundant protein in developing enamel. Proper processing and function is crucial for normal enamel development, as illustrated in AMEL gene knock out mice that have hypoplastic enamel. The main goal of this master thesis is to give an overview of amelogenin function and significance for normal enamel development with particular emphasis to celiac disease associated developmental enamel defects. Untreated celiac children have increased anti-gliadin and anti-AMEL antibodies presumably due to structural homologies. Thus, anti-gliadin antibodies may cross-react with AMEL and disturb normal enamel production. Approximately 40% of all celiacs have developmental enamel defects (DED). We will review the role amelogenin has in amelogenesis, and how celiac disease associated immune response to gliadin may cross-react to different AMEL peptides. We have illustrated this with an experiment that is part of a PhD degree project performed at the Institute of Oral Biology at the University of Oslo. Protein dot blots was used to detect IgA anti-AMEL immune reactivity in blood or serum from untreated celiac disease children. In particular, whether the anti-AMEL immune response included reactivity to the leucin rich amelogenin peptide (LRAP), suggested to be an important signaling peptide.