N-acetylgalactosamine-6-sulfatase is an enzyme that is encoded by the GALNS gene.
This gene encodes N-acetylgalactosamine-6-sulfatase which is a lysosomal exohydrolase required for the degradation of the glycosaminoglycans, keratan sulfate, and chondroitin 6-sulfate.
Sequence alterations including point, missense and nonsense mutations, as well as those that affect splicing, result in a deficiency of this enzyme.
Deficiencies of this enzyme lead to Morquio A syndrome, a lysosomal storage disorder.
Mucopolysaccharidosis type IVA is an autosomal recessive lysosomal storage disease characterized by intracellular accumulation of keratan sulfate and chondroitin-6-sulfate.
Key clinical features include short stature, skeletal dysplasia, dental anomalies, and corneal clouding.
Applications:Suitable for use in ELISA, Western Blot, Immunohistochemistry, Immunocytochemistry.
Other applications not tested.
Recommended Dilution:ELISA: 1:100-1:5000Western Blot: 1:50-400Immunohistochemistry (frozen): 1:50-500Immunohistochemistry (paraffin): 1:10-100Immunocytochemistry: 1:50-500Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：200ul
Host：rabbit
Source Antibody：rat
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
Specificity：Recognizes rat GAS.
Isotype：IgG
Calc Applications Abbrev：E IC IHC WB
Calc Crossreactivity：Rt
Immunogen：Recombinant protein corresponding to Asp172-Asp381of rat GAS, fused to two N-terminal Tags, His-Tag and T7-tag, expressed in E. coli.
