363553
IDUa (Iduronidase Alpha L, MPS1)
Size
£499.00
- SKU:
- 363553
- Purity:
- Purified by Protein A and peptide affinity chromatography
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Rabbit
- Immunogen:
- Recombinant protein corresponding to aa28-306 of human IDUa, expressed in E. coli
- Format:
- Supplied as a liquid in PBS, pH 7.4, 0.05% Proclin-300, 50% glycerol
- Extra Details:
- Iduronidase is an enzyme involved in the degeneration of glycosaminoglycans such as dermatan sulfate and heparan sulfate. It is found in the lysosomes of cells.Its deficiency is associated with mucopolysaccharidoses (MPS). MPS, a lysosomal storage disease, is typed I through VII. Type I is known as Hurler syndrome and type I,S is known as Scheie syndrome, which has a milder prognosis compared to Hurlers. The glycosaminoglycans accumulate in the lysosomes and cause substantial disease in many different tissues of the body. The defective alpha-L-iduronidase results in an accumulation of heparin and dermatin sulfate within phagocytes, endothelium, smooth muscle cells, neurons, and fibroblasts. Underneath electron microscopy these structure present as laminated structures, Zebra bodies. Applications: Suitable for use in ELISA, Western Blot, Immunohistochemistry and Immunocytochemistry. Other applications not tested. Recommended Dilution: Western Blot: 0.5-2ug/ml Immunohistochemistry (Paraffin): 5-20ug/ml Immunocytochemistry: 5-20ug/ml 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