Anti-NPC2 Antibody, clone 13G4

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.

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

GST-tagged recombinant human NPC2.

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

Research Category
Signaling

Research Sub Category
Vesicular 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).

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.

~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.

Protein G Purified

Format: Purified

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

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

Concentration: Please refer to lot specific datasheet.

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