Naglu active protein

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

Cat. Num. AAA638019

• Purity: Highly Purified; >95% by SDS-PAGE under reducing conditions
• Synonyms: NAGLU; Recombinant; Human (Alpha-N-acetylglucosaminidase; N-acetyl-alpha-glucosaminidase; NAG; UFHSD1); Naglu active protein

• Purity/Purification: Highly Purified; >95% by SDS-PAGE under reducing conditions
• Form/Format: Supplied as a liquid in 25mM Tris, 150mM sodium chloride. BSA free.
• Concentration: 0.88 mg/ml (varies by lot)

• 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)
• Preparation and Storage: 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.

Related Product Information for Naglu active protein

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.

Product Categories/Family for Naglu active protein

Molecular Biology; MB-Enzymes

NCBI and Uniprot Product Information

• NCBI GI #: 2660688
• NCBI GeneID: 27419
• UniProt Accession #: P54802
• Molecular Weight: ~81kD
• NCBI Official Full Name: Naglu
• NCBI Official Synonym Full Names: alpha-N-acetylglucosaminidase (Sanfilippo disease IIIB)
• NCBI Official Symbol: Naglu
• NCBI Protein Information: alpha-N-acetylglucosaminidase; N-acetyl-glucosaminidase; alpha-N-acetylglucosaminidase, lysosomal
• Protein Family: Alpha-N-acetylglucosaminidase

Uniprot Description

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