GAS (N-Acetylgalactosamine-6-Sulfatase, CS, GALNS, MPS4A, GALNAC6S, Chondroitin Sulfatase, Chondroitinase, Galactosamine(N-Acetyl)-6-Sulfate Sulfatase, Morquio Syndrome, Mucopolysaccharidosis Type IVA)

Größe

£495.00

SKU:: 363223
Reinheit:: Purified by Protein A and peptide affinity chromatography.
Lagerbedingungen:: -20°C
Hersteller:: United States Biological
Host:: Rabbit
Immunogen:: Recombinant protein corresponding to Asp172-Asp381of rat GAS, expressed in E. coli.
Format:: Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
Weitere Details:: 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 Western Blot and Immunohistochemistry. Other applications not tested. Recommended Dilution: Optimal 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.

Versandbedingungen:: Blue Ice