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Significance Lysosomes are cellular organelles containing a variety of degradative enzymes. The protein N-acetylglucosamine-1-phosphotransferase (GNPT) is responsible for directing these enzymes to be delivered to lysosomes. Genetic mutations in GNPT… Click to show full abstract
Significance Lysosomes are cellular organelles containing a variety of degradative enzymes. The protein N-acetylglucosamine-1-phosphotransferase (GNPT) is responsible for directing these enzymes to be delivered to lysosomes. Genetic mutations in GNPT cause the diseases mucolipidosis types II and III. In order to better understand the function of GNPT at the molecular level, we determined the three-dimensional structures of its catalytic domain and of its sugar-binding subunit. These structures, along with biochemical experiments, reveal its enzymatic mechanism and its sugar recognition mode.
Journal Title: Proceedings of the National Academy of Sciences of the United States of America
Year Published: 2022
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