{"product_id":"nsj-fy12863-100ug","title":"NAGLU Antibody \/ Alpha-N-acetylglucosaminidase, 100 ug","description":"\u003cp\u003eNAGLU antibody detects Alpha-N-acetylglucosaminidase, a lysosomal enzyme responsible for the degradation of heparan sulfate. Encoded by the NAGLU gene on chromosome 17q21.2, this hydrolase catalyzes the removal of N-acetylglucosamine residues from the non-reducing ends of heparan sulfate chains, playing a critical role in glycosaminoglycan catabolism. Proper function of NAGLU is essential for maintaining lysosomal homeostasis and preventing the accumulation of undegraded substrates.\u003cbr\u003e\u003cbr\u003eNAGLU is synthesized as a preproenzyme that undergoes proteolytic processing in the endoplasmic reticulum and Golgi before trafficking to lysosomes. The mature enzyme is approximately 83 kilodaltons and operates optimally at acidic pH. It functions in concert with other lysosomal hydrolases to complete stepwise degradation of heparan sulfate, ensuring normal turnover of extracellular matrix components and cell-surface proteoglycans.\u003cbr\u003e\u003cbr\u003eThe NAGLU antibody is widely used in enzymology, lysosomal biology, and metabolic disease research to study glycosaminoglycan metabolism, lysosomal trafficking, and enzyme replacement mechanisms. Western blot analysis identifies a band corresponding to the mature lysosomal form of NAGLU, while immunofluorescence shows vesicular staining overlapping with lysosomal markers such as LAMP1. This antibody enables precise detection of enzyme localization and expression changes in normal and diseased tissues.\u003cbr\u003e\u003cbr\u003eMutations in NAGLU cause mucopolysaccharidosis type IIIB (MPS IIIB), also known as Sanfilippo syndrome type B, a lysosomal storage disorder characterized by progressive neurodegeneration and systemic accumulation of heparan sulfate. The NAGLU antibody is a valuable tool for diagnosing and modeling this disease, assessing enzyme replacement efficacy, and monitoring lysosomal function in gene therapy studies. NSJ Bioreagents provides this antibody validated for its applications, ensuring high sensitivity for lysosomal and metabolic research.\u003c\/p\u003e","brand":"NSJ Bioreagents","offers":[{"title":"100 ug","offer_id":48537675268320,"sku":"FY12863","price":449.0,"currency_code":"KRW","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0601\/9330\/8896\/files\/get_image_c634cfdb-638d-44c7-b5eb-40ed0d220c93.jpg?v=1768353223","url":"https:\/\/rndmate.com\/products\/nsj-fy12863-100ug","provider":"알앤디메이트","version":"1.0","type":"link"}