NAGA is a lysosomal that cleaves non-reducinga -N-acetylgalactosaminyl moieties from glycoconjugates. Mature NAGA has 394aa and is trafficked to the lysosome via the mannose-6-phosphate receptor-mediated pathway. The enzyme is a retaining exoglycosidase, where both the substrate and product of the enzymatic reaction have the same anomeric configuration. Deficiency in NAGA results in increased urinary excretion and tissue accumulation of glycopeptides and oligosaccharides containing terminal a-N-acetylgalactosaminyl moieties, manifesting as Schindlers disease, an autosomal recessive disease with neuroaxonal dystrophy and other neurological symptoms. The enzyme can be used to remove a-N-acetylgalactosaminyl residues present on red blood cells thus converting blood type A to blood type O. Source: Recombinant corresponding to aa18-411 from human a-N-acetylgalactosaminyl fused to 6-His-tag and with an N-terminal CD33 signal peptide, expressed in Chinese Hamster Ovary cell line. Molecular Weight: ~46kD Biological Activity: Measured by its ability to cleave a-N-acetylgalactosaminyl from 4-Nitrophenyl N-acetyl-a-D-galactosaminide. Specific Activity: >1800pmol/min/ug Endotoxin: ~1EU/1ug (LAL) Storage and Stability: May be stored at 4C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20C. Aliquots are stable for 6 months at -20C. 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.
