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The UDP‐GlcNAc:lysosomal enzyme, N‐acetylglucosamine‐1‐phosphotransferase (GlcNAc‐1‐PT), is an α2β2γ2 hexamer that mediates the initial step in the formation of the mannose 6‐phosphate targeting signal on newly synthesized lysosomal acid hydrolases. The GNPTAB gene encodes the 1256 amino acid long α/β precursor which is normally cleaved at K928 in the early Golgi by Site‐1 protease (S1P). Here, we...
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