protein

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

MBS638019

0.02 mg

740 USD

Recombinant Protein

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

[NAGLU]

[Naglu; alpha-N-acetylglucosaminidase; N-acetyl-glucosaminidase; alpha-N-acetylglucosaminidase, lysosomal; alpha-N-acetylglucosaminidase (Sanfilippo disease IIIB)]

[Naglu]

Highly Purified. >95% by SDS-PAGE under reducing conditions

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

0.88 mg/ml

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 6 months after receipt at -20 degree 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.

Source: Recombinant, CHO cells. Specific Activity: >900pmol/min/ug. Biological Activity: Measured by its ability to hydrolyze 4-Nitrophenyl-N-acetyl-a-D-glucosaminide. Endotoxin Level: <1EU/1ug (LAL).

Molecular Biology; MB-Enzymes

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. <Recombinant protein corresponding to Met1-Trp743 from human NAGLU fused to 6-His-tag at C-terminal expressed in CHO cell line.

2660688

AAB88084.1

P54802

~81kD

Disease Pathway (819462); Glycosaminoglycan Degradation Pathway (83180); Glycosaminoglycan Degradation Pathway (355); Glycosaminoglycan Metabolism Pathway (819212); HS-GAG Degradation Pathway (819223); Heparan Sulfate Degradation Pathway (421777); Heparan Sulfate Degradation Pathway (468270); Heparan Sulfate/heparin (HS-GAG) Metabolism Pathway (819220); Lysosome Pathway (99272); Lysosome Pathway (96865)

NAGLU: Involved in the degradation of heparan sulfate. Defects in NAGLU are the cause of mucopolysaccharidosis type 3B (MPS3B); also known as Sanfilippo syndrome B. MPS3B is a form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. Protein type: Glycan Metabolism - glycosaminoglycan degradation; EC 3.2.1.50; Hydrolase. Molecular Function: alpha-N-acetylglucosaminidase activity. Biological Process: locomotor rhythm; retinal rod cell development; lysosome organization and biogenesis; inner ear receptor cell development; cerebellar Purkinje cell layer development; middle ear morphogenesis

0.02 mg

740 USD