GlcNAc-1-Phosphotransferase
GlcNAc-1-Phosphotransferase is a Golgi membrane-bound glycosylphosphotransferase. GlcNAc-1-Phosphotransferase is composed of an α/β catalytic subunit encoded by the GNPTAB gene and a γ regulatory subunit encoded by the GNPTG gene, forming an α2β2γ2 hexameric complex. GlcNAc-1-Phosphotransferase catalyzes the transfer of GlcNAc-1-phosphate from UDP-GlcNAc to the C-6 hydroxyl group of terminal mannose residues on high-mannose-type N-glycans, thereby initiating the mannose-6-phosphate tagging pathway. GlcNAc-1-Phosphotransferase loss-of-function mutations can lead to lysosomal storage diseases such as mucolipidosis type II/III. GlcNAc-1-Phosphotransferase can be used in research related to mucolipidosis.
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- CAS No.: 84012-69-1
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Biological Activity
Description
In Vitro
GlcNAc-1-Phosphotransferase utilizes Man6GlcNAc2 for monophosphorylation and Man7GlcNAc2 for bisphosphorylation as its major M6P glycan substrates in mouse tissues[2].
GlcNAc-1-Phosphotransferase's Drosophila homolog DmGNPTAB was expressed and purified in insect cells, and its homodimeric structure was determined by cryo-electron microscopy at 3.5 Å resolution, revealing a catalytic domain formed by the folding of four conserved regions (CR1-CR4) and a putative UDP-GlcNAc binding pocket[3].
GlcNAc-1-Phosphotransferase catalytic site mutants such as N406A, D408A, and C1149A failed to fully restore CatB maturation in GNPTAB-/- HeLa cells, among which D408A was completely inactive, demonstrating that these residues are essential for enzyme activity[3].
GlcNAc-1-Phosphotransferase is functionally lost in CRISPR-Cas9-mediated GNPTAB-/- HeLa cells, leading to lysosomal swelling and impaired maturation of cathepsin B (CatB), with the mature form of CatB being undetectable, indicating severe lysosomal dysfunction[3].
MedChemExpress (MCE) has not independently confirmed the accuracy of these methods. They are for reference only. Further protocols information, click here.
Chemical Information
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CAS No. 84012-69-1
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SMILES
[GlcNAc-1-Phosphotransferase]
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Storage
Please store the product under the recommended conditions in the Certificate of Analysis.
Protocols
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Lysosome and acidic-vesicle live-cell staining
Lysosome and acidic-vesicle live-cell staining detects acidic intracellular compartments by using membrane-permeant acidotropic probes that accumulate in low-pH vesicles, including lysosomes, late endosomes, autolysosomes, and acidic phagosomes. LysoTracker staining is commonly used as an intensity-based readout of acidic lysosomal compartment abundance or enlargement, while acridine orange produces green fluorescence in less concentrated compartments and red fluorescence after concentration-dependent accumulation in acidic vesicular organelles. Loss or reduction of acridine-orange red signal can be used as a readout of lysosomal membrane permeabilization or reduced acidic-vesicle integrity. This protocol is designed for live cultured cells and can be adapted for fluorescence microscopy, high-content imaging, plate-reader readout, or flow cytometry when the selected literature supports the readout. Because these dyes report acidotropic accumulation rather than lysosome identity alone,
Purity & Documentation
References
Calculators
Concentration (start) × Volume (start) = Concentration (final) × Volume (final)
GlcNAc-1-Phosphotransferase Related Classifications
HY-E70305 Related Classifications
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