Anti-NAGLU Antibody Picoband®

SKU: A03549

Size: 100 μg

Storage: At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat
ELISA, 0.1-0.5 μg/ml

Gene Name: NAGLU

Specificity:

Background: N-acetylglucosaminidase, alpha is a protein that in humans is encoded by the NAGLU gene. This gene encodes an enzyme that degrades heparan sulfate by hydrolysis of terminal N-acetyl-D-glucosamine residues in N-acetyl-alpha-D-glucosaminides. Defects in this gene are the cause of mucopolysaccharidosis type IIIB (MPS-IIIB), also known as Sanfilippo syndrome B. This disease is characterized by the lysosomal accumulation and urinary excretion of heparan sulfate.

Immunogen: E.coli-derived human NAGLU recombinant protein (Position: D24-D489).

Clonality: Polyclonal

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

Purification: Immunogen affinity purified.

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

Reference: 1. Beesley, C. E., Young, E. P., Vellodi, A., Winchester, B. G. Identification of 12 novel mutations in the alpha-N-acetylglucosaminidase gene in 14 patients with Sanfilippo syndrome type B (mucopolysaccharidosis type IIIB). J. Med. Genet. 35: 910-914, 1998. 2. Bunge, S., Knigge, A., Steglich, C., Kleijer, W. J., van Diggelen, O. P., Beck, M., Gal, A. Mucopolysaccharidosis type IIIB (Sanfilippo B): identification of 18 novel alpha-N-acetylglucosaminidase gene mutations. J. Med. Genet. 36: 28-31, 1999. 3. Chinen, Y., Tohma, T., Izumikawa, Y., Uehara, H., Ohta, T. Sanfilippo type B syndrome: five patients with an R565P homozygous mutation in the alpha-N-acetylglucosaminidase gene from the Okinawa islands in Japan. J. Hum. Genet. 50: 357-359, 2005.

Uniprot ID: P54802

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 82 kDa

Calculated Molecular Weight: 82 kDa

Gene ID: 4669

Protein Name: Alpha-N-acetylglucosaminidase

Gene Full Name: N-acetyl-alpha-glucosaminidase

Synonyms: NAGLU; UFHSD1; Alpha-N-acetylglucosaminidase; EC 3.2.1.50; N-acetyl-alpha-glucosaminidase; NAG [Cleaved into: Alpha-N-acetylglucosaminidase 82 kDa form; Alpha-N-acetylglucosaminidase 77 kDa form]