364405
NPC2 (Niemann Pick Disease Type C2, HE1, NP-C2, EDDM1, Epididymal Protein 1, Human epididymis-specific protein 1, Epididymal secretory protein E1)
Size
£517.00
- SKU:
- 364405
- Application:
- WB
- Purity:
- Purified by Protein A and peptide affinity chromatography.
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Rabbit
- Immunogen:
- Recombinant protein corresponding to aa20-151 of human NPC2, expressed in E. coli.
- Format:
- Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
- Extra Details:
- Mutations in the NPC2 gene associated with Niemann-Pick disease type C2 cause abnormally high cholesterol accumulation in cells. NPC2, a secreted soluble protein, binds cholesterol specifically with a much higher affinity than previously reported. Genetic and biochemical studies identified single amino acid changes that prevent both cholesterol binding and the restoration of normal cholesterol levels in mutant cells. The amino acids that affect cholesterol binding surround a hydrophobic pocket in the NPC2 protein structure, identifying a candidate sterol-binding location. On the basis of evolutionary analysis and mutagenesis, 3 other regions of the NPC2 protein emerged as important, including 1 required for efficient secretion. Applications: Suitable for use in Western Blot. 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.
- Shipping Conditions:
- Blue Ice