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| Reactivity: | Human |
| Applications: | WB, ELISA |
| Host Species: | Rabbit |
| Clonality: | Polyclonal antibody |
| Gene Name: | N-acetylglucosamine-1-phosphate transferase subunits alpha and beta |
| Gene Symbol: | GNPTAB |
| Synonyms: | ICD; GNPTA; GNPTAB |
| Gene ID: | 79158 |
| UniProt ID: | Q3T906 |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 42-350 of human GNPTAB (NP_077288.2). |
| Dilution: | WB 1:500-1:2000 |
| Purification Method: | Affinity purification |
| Concentration: | 1.12 mg/ml |
| Buffer: | Buffer: PBS with 0.01% thimerosal,50% glycerol,pH7.3. |
| Storage: | Store at -20°C. Avoid freeze/thaw cycles. |
| Documents: | Manual-GNPTAB antibody |
Background
This gene encodes two of three subunit types of the membrane-bound enzyme N-acetylglucosamine-1-phosphotransferase, a heterohexameric complex composed of two alpha, two beta, and two gamma subunits. The encoded protein is proteolytically cleaved at the Lys928-Asp929 bond to yield mature alpha and beta polypeptides while the gamma subunits are the product of a distinct gene (GeneID 84572). In the Golgi apparatus, the heterohexameric complex catalyzes the first step in the synthesis of mannose 6-phosphate recognition markers on certain oligosaccharides of newly synthesized lysosomal enzymes. These recognition markers are essential for appropriate trafficking of lysosomal enzymes. Mutations in this gene have been associated with both mucolipidosis II and mucolipidosis IIIA.
Images
 | Western blot analysis of various lysates using GNPTAB Rabbit pAb (A15895) at 1:1000 dilution. Secondary antibody: HRP-conjugated Goat anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution. Lysates/proteins: 25μg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Enhanced Kit (RM00021). Exposure time: 90s. |
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