OAT-2068 is a selective, high activity and orally active inhibitor of mouse chitotriosidase (mCHIT1) ( IC 50 =29 nM) and displays 143-fold selectivity over m AMCase ( IC 50 =4170 nM). OAT-2068 displays a good pharmacokinetic profile and is an ideal tool to study the role of CHIT1 in biological systems, including animal models of human diseases.