362041
AGA (Aspartylglucosaminidase, AGU, ASRG, GA, N(4)-(beta-N-acetylglucosaminyl)-L-asparaginaseGlycosylasparaginase, N4-(N-acetyl-beta-glucosaminyl)-L-asparagine amidase)
Size
£626.00
- SKU:
- 362041
- Purity:
- Purified by immunoaffinity chromatography.
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Rabbit
- Immunogen:
- Recombinant protein corresponding to Pro28-Ala245 of human AGA,, expressed in E. coli.
- Format:
- Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
- Extra Details:
- Aspartylglucosaminidase is an amidohydrolase enyzme involved in the catabolism of N-linked oligosaccharides of glycoproteins. It cleaves asparagine from N-acetylglucosamines as one of the final steps in the lysosomal breakdown of glycoproteins. The lysosomal storage disease aspartylglycosaminuria is caused by a deficiency in the AGA enzyme. Defects in AGA are the cause of aspartylglucosaminuria (AGU). AGU is an inborn lysosomal storage disease. Clinical features of AGU include mild to severe mental retardation manifesting from the age of 2, coarse facial features and mild connective tissue abnormalities. This recessively inherited disease is overrepresented in the Finnish population. Applications: Suitable for use in ELISA, Western Blot, Immunohistochemistry, Immunocytochemistry. Other applications not tested. Recommended Dilution: ELISA: 1:100-1:5000 Western Blot: 1:50-400 Immunohistochemistry (frozen): 1:50-500 Immunohistochemistry (paraffin): 1:10-100 Immunocytochemistry: 1:50-500 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.
- Shipping Conditions:
- Blue Ice