NAGLU anticorps (AA 24-489)

N° du produit ABIN7872697

Cet anticorps anti-NAGLU Polyclonal Lapin (ABIN7872697) détecte spécifiquement NAGLU dans WB et ELISA. L’anticorps est réactif avec des échantillons de Souris, Humain et Rat.

Aperçu rapide pour NAGLU anticorps (AA 24-489) (ABIN7872697)

Antigène: NAGLU (N-Acetylglucosaminidase, alpha (NAGLU))

Reactivité: Souris, Humain, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp NAGLU est non-conjugé

Application: Western Blotting (WB), ELISA

Détail du produit anti-NAGLU anticorps

Épitope: AA 24-489

Fonction: NAGLU Antibody / Alpha-N-acetylglucosaminidase

Purification: Immunogen affinity purified

Immunogène: E.coli-derived human NAGLU recombinant protein (Position: D24-D489) was used as the immunogen for the NAGLU antibody.

Isotype: IgG

Information d'application

Indications d'application: Optimal dilution of the NAGLU antibody should be determined by the researcher.

Restrictions: For Research Use only

Stockage

Format: Lyophilized

Reconstitution: Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Stock: 4 °C,-20 °C

Stockage commentaire: After reconstitution, the NAGLU antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.

Détails sur NAGLU

Antigène: NAGLU (N-Acetylglucosaminidase, alpha (NAGLU))

Autre désignation: NAGLU

Sujet: NAGLU 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.

NAGLU 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.

The 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.

Mutations 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.

UniProt: P54802

Pathways: Glycosaminoglycan Metabolic Process