Detection of Human WFS1 by Western Blot.
Western blot shows lysates of IMR‑32 human neuroblastoma cell line. PVDF membrane was probed with 1 µg/mL of Sheep Anti-Human WFS1 Antigen Affinity-purified Polyclonal Antibody (Catalog # AF7417) followed by HRP-conjugated Anti-Sheep IgG Secondary Antibody (Catalog # HAF016). A specific band was detected for WFS1 at approximately 100 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 8.
Preparation and Storage
Sterile PBS to a final concentration of 0.2 mg/mL.
Reconstitution Buffer Available
The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below. *Small pack size (SP) is shipped with polar packs. Upon receipt, store it immediately at -20 to -70 °C
Stability & Storage
Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
12 months from date of receipt, -20 to -70 °C as supplied.
1 month, 2 to 8 °C under sterile conditions after reconstitution.
6 months, -20 to -70 °C under sterile conditions after reconstitution.
WFS1 (Wolframin Syndrome gene 1; also Wolframin) is a 100-105 kDa intracellular glycoprotein that contains an unusual eleven transmembrane (TM) topology. It is widely expressed, being found in neurons, fibroblasts, hepatocytes, stratified squamous epithelium and pancreatic beta -cells. WFS1 is found in the ER and select secretory vesicles. It is known to be induced by ER stress, which prompts it to increase Ca++ in the ER, a condition necessary for proper protein folding. It also contributes to the maintenance of the proper pH in insulin-containing granules. Human WSF1 is 890 amino acids (aa) in length. It is a type II 11-TM protein that possesses a cytoplasmic N-terminus (aa 1-313) and transmembrane-embedded C-terminus (aa 870-890). WSF1 is reported to form homodimers and homotetramers. There are multiple mutations in the WFS1 gene that contribute to Wolfram syndrome. Among these are an isoform that generates a premature truncation at Ser157, a second isoform that possesses a seven aa substitution for aa 509-890, and a third isoform which shows a deletion of aa 508-512. Over aa 679-783, human WFS1 shares 95% aa sequence identity with mouse WFS1.
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