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N-acetylglucosamine-1-phosphate transferase

Mammalian protein found in Homo sapiens From Wikipedia, the free encyclopedia

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N-acetylglucosamine-1-phosphate transferase (GlcNAc-1-phosphotransferase) is a transferase enzyme.

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Function

It is made up of two alpha (α), two betas (β), and two gammas (γ) subunits. GNPTAB produces the alpha and beta subunits, GNPTG produces the gamma subunit. GlcNAc-1-phosphotransferase functions to prepare newly made enzymes for lysosome transportation (lysosomal hydrolases to the lysosome). Lysosomes, a part of an animal cell, helps break down large molecules into smaller ones that can be reused. GlcNAc-1-phosphotransferase phosphorylates carbon 6 of one or more mannosyl residues of N linked glycoproteins being processed in Golgi Apparatus . UDP-GLcNAc provides the phosphate in a reaction catalysed by this enzyme. M6P acts as an indicator of whether a hydrolase should be transported to the lysosome or not. Once a hydrolase indicates an M6P, it can be transported to a lysosome. Surprisingly some lysosomal enzymes are only tagged at a rate of 5% or lower.

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Clinical significance

It is associated with the following conditions:[1][2]

In melanocytic cells, GNPTG gene expression may be regulated by MITF.[3]

References

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