Product Datasheet  
GNPTAB Polyclonal Antibody  
Catalog Number: 29478  
Technical:tech@swbio.com  
Information:info@swbio.com  
Description  
  • host_species:  
  • Rabbit
  • Amount:  
  • 100μgμg
  • Swiss-Prot No.:  
  • Swiss-Prot#:Q3T906
    NCBI Gene ID:79158
  • Form of Antibody:  
  • Avoid freeze / thaw cycles.|Buffer: PBS with 50% glycerol, pH7.4.
  • Storage:  
  • Store at -20˚C
  • Immunogen:  
  • Recombinant fusion protein of human GNPTAB (NP_077288.2).
  • reactivity:  
  • Human
  • appl_detail:  
  • WB 1:500 - 1:2000
  • other_names:  
  • GNPTAB; GNPTA; ICD; N-acetylglucosamine-1-phosphotransferase subunits alpha/beta
  • Purification:  
  • Affinity purification
  • Specificity:  
  • Applications:  
  • WB
  • 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.



 
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