NAGLU
NAGLU (N-acetyl-alpha-glucosaminidase, also known as NAG) is a lysosomal glycoside hydrolase involved in the degradation of heparan sulfate. Defects in the NAGLU gene cause mucopolysaccharidosis type IIIB, also called Sanfilippo syndrome B. Browse Assay Genie's current range of NAGLU research reagents below, or read on for the biology behind the target.
NAGLU encodes alpha-N-acetylglucosaminidase, an enzyme that degrades heparan sulfate. It does so by hydrolysing terminal N-acetyl-D-glucosamine residues in N-acetyl-alpha-D-glucosaminides, a step in the stepwise breakdown of this glycosaminoglycan. The protein is found in the lysosome, the compartment where this catabolic work takes place.
The NAGLU protein belongs to the glycosyl hydrolase 89 family, and HGNC places the gene in the wider glycoside hydrolase family group. The enzyme is classified under EC 3.2.1.50. Researchers working on this enzyme may look for a NAGLU antibody, a NAGLU ELISA kit or recombinant NAGLU protein to study its expression, activity and handling in lysosomal biology.
The gene is of particular interest because of its disease association. Defects in the NAGLU gene are the cause of mucopolysaccharidosis type IIIB (MPS-IIIB), also known as Sanfilippo syndrome B. The disease is characterised by the lysosomal accumulation and urinary excretion of heparan sulfate, which reflects the loss of the enzyme's degradative function. Previous names for the gene include N-acetylglucosaminidase, alpha, and NAG remains a common alias in the literature.
Loss of NAGLU function causes mucopolysaccharidosis type IIIB, making it a central target in studies of lysosomal storage disease.
NAGLU hydrolyses terminal N-acetyl-D-glucosamine residues, so it is used to study how heparan sulfate is degraded.
Defects in NAGLU lead to lysosomal heparan sulfate accumulation and its urinary excretion, hallmarks of Sanfilippo syndrome B.
As a member of the glycosyl hydrolase 89 family, NAGLU is a model for studying glycoside hydrolase function.
NAGLU is a lysosomal enzyme involved in the degradation of heparan sulfate. It hydrolyses terminal N-acetyl-D-glucosamine residues in N-acetyl-alpha-D-glucosaminides, allowing this glycosaminoglycan to be broken down within the lysosome.
NAGLU is the HGNC symbol for N-acetyl-alpha-glucosaminidase. UniProt calls the protein alpha-N-acetylglucosaminidase, NAG is a listed alias, and a previous gene name was N-acetylglucosaminidase, alpha. Check the datasheet of any product for its validated reactivity.
Defects in NAGLU cause mucopolysaccharidosis type IIIB (MPS-IIIB), also called Sanfilippo syndrome B. The disorder involves lysosomal accumulation and urinary excretion of heparan sulfate, consistent with the enzyme's role in degrading it.
Other lysosomal enzymes and glycoside hydrolases involved in glycosaminoglycan breakdown may be of interest alongside NAGLU.
Gene and protein annotations from HGNC, NCBI Gene and UniProt P54802; product specifications from Assay Genie datasheets.