Unexpected Roles of Exostosin-like 2, EXTL2, in Glycosaminoglycan Biosynthesis and Function

抄録

In 1999, I received the Young Scientist Award from the Japanese Society of Carbohydrate Research for my research leading to the identification of the tumor suppressor EXT-like gene family member EXTL2. Specifically, I identified an N-acetylhexosaminyltransferase that transfers not only N-acetylgalactosamine but also N-acetylglucosamine to the glycosaminoglycan-protein linkage region. Since that award, my laboratory has shown that EXTL2 functions as a suppressor of glycosaminoglycan biosynthesis and that this suppression is enhanced by the xylose kinase FAM20B. Furthermore, we have found that an EXTL2-dependent mechanism that regulates glycosaminoglycan biosynthesis is important for maintaining tissue homeostasis under pathological conditions, and that lack of EXTL2 causes glycosaminoglycan overproduction and structural changes in glycosaminoglycans associated with pathological processes.