(800) 943-6396

150 Essex St, Millburn, NJ 07041, USA

©2019 by Bon Opus Biosciences, LLC.

Niemann Pick C1 Recombinant Rabbit monoclonal Antibody IgG

SKU: BA112529-100µl
$279.00Price

Fig1: Western blot analysis of Niemann Pick C1 on SiHa cell lysate using anti-Niemann Pick C1 antibody at 1/500 dilution.

Fig2: ICC staining Niemann Pick C1 in A549 cells (green). The nuclear counter stain is DAPI (blue). Cells were fixed in paraformaldehyde, permeabilised with 0.25% Triton X100/PBS.

Fig3: ICC staining Niemann Pick C1 in HepG2 cells (green). The nuclear counter stain is DAPI (blue). Cells were fixed in paraformaldehyde, permeabilised with 0.25% Triton X100/PBS.

Bon Opus Cat. #BA112529
Size
  • Host Species; Species Reactivity

    Rabbit; Human, Mouse, Rat
  • Immunogen

    Recombinant protein
  • Application Summary

    WB,ICC,IF,IHC,FC
  • Purification; Formulation

    ProA affinity purified; 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.; Liquid form.
  • ALTnames

    NPC intracellular cholesterol transporter 1, Niemann-Pick C1 protein
  • Background

    Cells obtain cholesterol via two distinct pathways, endogenous synthesis in the endoplasmic reticulum and exogenous uptake through the low-density lipoprotein (LDL) receptor pathway. NPC1 is a protein that resides in late endosomes and lysosomes and is involved in the intracellular trafficking of cholesterol. The human NPC1 gene maps to chromosome 18q11.2 and produces proteins which undergo N-glycosylation and are expressed in brain and liver. NPC1 contains a cysteine-rich domain, which is critical for proper protein function, but is highly mutated. Mutations in NPC1 result in Niemann-Pick disease type C (NPC), an autosomal recessive disease characterized by the accumulation of unesterified cholesterol in the endosomal/lysosomal system. The accumulation of cholesterol results in progressive neurodegeneration and death. More than 90% of cases of NPC are due to mutations in NPC1 and patients with NPC display multiple neurological symptoms, such as hepatosplenomegaly, ataxia, dystonia and dementia.(ET7107-57)