NAGLU, Recombinant, Human (Alpha-N-acetylglucosaminidase, N-acetyl-alpha-glucosaminidase, NAG, UFHSD1)

Human lysosomal A-N-acetylglucosaminidase is a hydrolase that catalyses the removal of terminal A-N-acetylglucosamine residues from heparan sulfate and heparin. Defects in this gene are the cause of mucopolysaccharidosis type IIIB (MPS-IIIB), also known as Sanfilippo syndrome B. Mucopolysaccharidosis types IIIA, C, and D are caused by mutations in other genes involved in the lysosomal degradation of heparan sulfate. Continuous lysosomal accumulation of heparan sulfate results in the clinical onset of disease, which is typified by severe central nervous system degeneration. Mucopolysaccharidosis type III differs from other mucopolysaccharidoses in that patients usually exhibit mild somatic changes with minimal skeletal abnormalities.\n\nRecombinant protein corresponding to aa1-743 from human NAGLU fused to 6-His-tag at C-terminal expressed in CHO cell line.\n\nMolecular Weight:\n~81kD\n\nSpecific Activity:\n~900pmol/min/ug\n\nBiological Activity:\nMeasured by its ability to hydrolyze 4-Nitrophenyl-N-acetyl-a-D-glucosaminide.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for 6 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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SPECIFICATIONS

Catalog Number

N0017-06

Size

20ug

Form

Supplied as a liquid in 25mM Tris, 150mM sodium chloride. BSA free.

Purity

~95% by SDS-PAGE under reducing conditions\nEndotoxin: ~1EU/1ug (LAL)

References

1. Yogalingam, G. et al. (2000) Biochim. Biophy. Acta 1502:415. 2. Zhao, H.G. et al. (1996) Proc. Natl. Acad. Sci. U.S.A. 93:6101. 3. Schmidtchen, A. et al. (1998) Am. J. Hum. Genet. 62:64. 4. Weber, B. et al. (1999) Eur. J. Hum. Genet. 7:34. 5. Beesley, C.E. et al. (2005) J. Inherit. Metab. Dis. 28:759. 6. Yogalingam, G. and Hopwood, J.J. (2001) Hum. Mutat. 18:264.