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Exostosin-like 2, or EXTL2 can be found in the extracellular region of the endoplasmic reticulum and is integral to the membrane. Belonging to the glycosyltransferase 47 family, EXTL2 encodes 4-N-acetylhexosamynyltransferase and transfers N-acetylglucosamine to the glycosaminoglycan (GAG) protein linkage region. More specifically, EXTL2 is critical for the synthesis of heparin/heparan sulfate, and the lack of EXTL2 can cause GAG overproduction. Biological processes associated with EXTL2 include the N-acetylglucosamine metabolic process, heparan sulfate proteoglycan biosynthetic process and UDP-N-acetylgalactosamine metabolic process. Known diseases for EXTL2 include Exostosis and hereditary multiple exostoses.
