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Simplified scheme of M6P-dependent enzymes sorting to the lysosome. The enzyme UDP-N-acetylglucosamine-1-phosphotransferase, responsible for the initial step in the synthesis of the M6P recognition markers, plays a key role in lysosomal enzyme trafficking. Loss of this activity results in mucolipidoses II/III. Note that not all lysosomal enzymes depend on the M6P pathway.
