N-acetylglucosamine-1-phosphotransferase subunits alpha/beta

Definition:

Catalyzes the formation of mannose 6-phosphate (M6P) markers on high mannose type oligosaccharides in the Golgi apparatus. M6P residues are required to bind to the M6P receptors (MPR), which mediate the vesicular transport of lysosomal enzymes to the endosomal/prelysosomal compartment.

References:

[1]. Yi Qian, et al. Functions of the alpha, beta, and gamma subunits of UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase. J Biol Chem. 2010 Jan 29;285(5):3360-70. [Content Brief]

[2]. Yi Qian, et al. The DMAP interaction domain of UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase is a substrate recognition module. Proc Natl Acad Sci U S A. 2013 Jun 18;110(25):10246-51. [Content Brief]

[3]. Nataniel Floriano Ludwig, et al. GNPTAB missense mutations cause loss of GlcNAc-1-phosphotransferase activity in mucolipidosis type II through distinct mechanisms. Int J Biochem Cell Biol. 2017 Nov;92:90-94. [Content Brief]

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