NAGLU Antikörper (AA 24-489)

Produktnummer ABIN7872697

Der Kaninchen Polyklonal anti-NAGLU Antikörper (ABIN7872697) detektiert spezifisch NAGLU in WB und ELISA. Dieser Antikörper reagiert spezifisch mit Proben aus Maus, Human und Ratte.

Kurzübersicht für NAGLU Antikörper (AA 24-489) (ABIN7872697)

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

Reaktivität: Maus, Human, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser NAGLU Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA

Produktdetails anti-NAGLU Antikörper

Bindungsspezifität: AA 24-489

Verwendungszweck: NAGLU Antibody / Alpha-N-acetylglucosaminidase

Aufreinigung: Immunogen affinity purified

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

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: Optimal dilution of the NAGLU antibody should be determined by the researcher.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Rekonstitution: 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.

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: 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.

Details zu NAGLU

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

Andere Bezeichnung: NAGLU

Hintergrund: 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