General Notes
This product (Catalog No. DPABH-00727) is a rabbit-derived polyclonal antibody raised against human NAGLU, targeting the amino acid region 485–743 of the protein. This antibody exhibits cross-reactivity with Human NAGLU from mouse and rat. It has been affinity purified by Protein A. NAGLU is a human gene that encodes the lysosomal enzyme alpha-N-acetylglucosaminidase, which catalyzes the hydrolysis of terminal N-acetyl-D-glucosamine residues from heparan sulfate chains, facilitating the breakdown of this glycosaminoglycan within lysosomes. This enzyme is essential for proper lysosomal function and the catabolism of heparan sulfate, a component of proteoglycans found in extracellular matrices and on cell surfaces. Mutations in the NAGLU gene lead to deficient enzyme activity, resulting in the accumulation of undegraded heparan sulfate and causing Sanfilippo syndrome type B (mucopolysaccharidosis type IIIB), an autosomal recessive lysosomal storage disorder characterized by severe neurological deterioration, behavioral abnormalities, and progressive somatic features. The gene is located on chromosome 17q21.2 and spans approximately 8 kb, with its protein product consisting of 743 amino acids and exhibiting optimal activity at acidic pH levels typical of lysosomal environments.
DPABH-00727 is suitable for use in ELISA (1:4,000–1:8,000), IHC (1:50–1:100), Western blot (WB) (1:1,000–1:4,000) applications.
DPABH-00727 is supplied as a liquid formulation in 0.01M PBS, pH 7.4, 50% Glycerol, 0.05% Proclin 300. For long-term storage, keep at −20 °C. For short-term use, store at 2–8 °C. Avoid repeated freeze–thaw cycles to maintain antibody integrity and performance.
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