Human Glucosamine (N-acetyl)-6‑Sulfatase/GNS Alexa Fluor® 594-conjugated Antibody

Applications

Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.

Preparation and Storage

Background: Glucosamine (N-acetyl)-6-Sulfatase/GNS

A member of the sulfatase family, GNS is required for the lysosomal degradation of the glycosaminoglycans (GAG) heparan sulfate and keratan sulfate (1, 2). It hydrolyzes the 6-sulfate group of the N-acetyl-D-glucosamine 6-sulfate units of the GAG. GNS deficiency results in mucopolysaccharidosis type IIID (MPS IIID or Sanfilippo D Syndrome), an inborn error leading to lysosomal accumulation of heparan sulfate. MPS IIID has profound mental deterioration, hyperactivity, and relatively mild somatic manifestations. The deduced amino acid sequence of human GNS consists of a signal peptide (residues 1‑36) and a mature chain (residues 37‑552) that may be further processed into N-terminal and C-terminal fragments (3). Recombinant human GNS corresponds to the single chain and has sulfatase activity.

Product Datasheets

Related Research Areas

• Glycobiology-related Enzymes
• Lysosomal Enzymes
• Proteoglycan Regulators
• Search Glycobiology-related Enzymes
• Sulfation Regulators