Application

Research CategorySignaling

Research Sub CategoryVesicular Trafficking

Western Blotting Analysis: A representative lot detected the intracellular organelle protein NPC2 in whole cell lysates, but not in plasma membrane extracts, from SV-589 human skin fibroblasts (Das, A., et al. (2013). Proc. Natl. Acad. Sci. U. S. A. 110(26):10580-10585).

Anti-NPC2 Antibody, clone 13G4 is an antibody against NPC2 for use in Western Blotting.

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

General description

Epididymal secretory protein E1 (UniProt P61916; also known as Human epididymis-specific protein 1, He1, Niemann-Pick disease type C2 protein) is encoded by the NPC2 (also known as HE1) gene (Gene ID 10577) in human. Niemann-Pick C1 & C2 (NPC1 & NPC2) proteins are involved in the transport of cholesterol and other lipids out of late endosomes and lysosomes. NPC1 is a large membrane spanning glycoprotein mainly located in the late endosomes, while NPC2 is a small soluble protein that binds nonesterified cholesterol with high affinity. NPC1 and NPC2 defects affect the intracellular trafficking of unesterified cholesterol and other lipids, causing the rare autosomal recessive lysosomal storage disorder known as Niemann-Pick type C disease. The majority of NPC patients have a defect in NPC1 (95%), while only 5% of the NPC cases are due to mutations in NPC2.

Immunogen

GST-tagged recombinant human NPC2.

Other Notes

Concentration: Please refer to lot specific datasheet.

Physical form

Purified mouse monoclonal IgG1κ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Protein G Purified

Format: Purified

Quality

Evaluated by Western Blotting in HT1080 human fibrosarcoma cell lysate.

Western Blotting Analysis: 0.5 µg/mL of this antibody detected NPC2 in 10 µg of HT1080 human fibrosarcoma cell lysate.

Specificity

Clone 13G4 detects Niemann-Pick disease type C2 protein (NPC2). Reactivity toward the spliced isoform 2 (UniProt P61916-2) has not been determined.

Storage and Stability

Stable for 1 year at 2-8°C from date of receipt.

Target description

~17 kDa observed. 16.57 kDa (isoform 1) and 13.42 kDa (isoform 2) calculated. A broad, smeared banding pattern in the range from ~17 to 25 kDa is typically seen.