Abcam, ab154508, Anti-NAGLU/NAG antibody

Size: 100µL
Rabbit Polyclonal NAGLU/NAG antibody. Suitable for IHC-P, WB, ICC/IF and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human NAGLU aa 250-500.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:WB, IHC-P, ICC/IFSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human NAGLU aa 250-500. The exact immunogen used to generate this antibody is proprietary information.P54802

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Immunogen, Storage buffer-pH: 7Preservative: 0.01% Thimerosal (merthiolate)Constituents: 20% Glycerol (glycerin, glycerine), 1.21% Tris, 0.75% Glycine, Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
NAGLU/NAG also known as alpha-N-acetylglucosaminidase is an enzyme with a molecular weight of approximately 83 kDa. This enzyme localizes in the lysosome where cells express it across various tissues. NAGLU plays a significant role in the breakdown of heparan sulfate which is a glycosaminoglycan found on cell surfaces and in the extracellular matrix. Its enzymatic action involves the cleavage of terminal alpha-N-acetylglucosamine residues during lysosomal degradation.
Biological function summary
NAGLU facilitates the degradation of heparan sulfate in conjunction with other enzymes as part of a degradation complex within the lysosome. NAGLU's action is essential for maintaining the balance of heparan sulfate levels inside cells which is important for cellular health and function. Without the proper function of this enzyme heparan sulfate accumulates within lysosomes due to incomplete catabolism leading to cellular dysfunction and related pathologies.
Pathways
NAGLU functions critically in the glycosaminoglycan degradation pathway. This pathway involves several enzymes including iduronate-2-sulfatase which acts upstream of NAGLU. Together they ensure the breakdown of complex sugars such as heparan sulfate. NAGLU also participates in the larger lysosomal degradation pathway collaborating with other lysosomal enzymes to ensure proper breakdown and recycling of cellular materials.
Disruptions in NAGLU function are directly linked to mucopolysaccharidosis type IIIB (MPS IIIB) also known as Sanfilippo syndrome type B. This disorder results from mutations that impair NAGLU activity causing heparan sulfate accumulation and neurodegeneration. Additionally deficient NAGLU activity may intersect with pathologies involving heparan sulfate binding proteins impacting cellular signaling and contributing to disease progression.