Niemann Pick C1 Polyclonal Antibody
Sizes: 50µl, 100µl
Catalogue Numbers: BS90945-50, BS90945-100
Product: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Swiss-Prot: O15118(Human) O35604(Mouse)
Host: Rabbit
Reactivity: Human, Mouse, Rat
Applications: WB, ICC, IF, IHC, FC
All Applications: WB:1:500-1:1,000
ICC/IF:1:50-1:200
IHC:1:50-1:200
FC:1:50-1:100
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.
Purification and Purity: ProA affinity purified
Storage and Stability: Store at +4°C after thawing. Aliquot store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Specificity: Niemann Pick C1 polyclonal antibody detects endogenous levels of Niemann Pick C1 protein.
Bioworld Molecular Weight: 142 kDa
Note: For research use only, not for use in diagnostic procedure.
Extra Notes: Western blot analysis of Niemann Pick C1 on SiHa cell lysate using anti-Niemann Pick C1 antibody at 1/500 dilution., 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.
Alternative Name: NPC1, Niemann Pick C1 protein precursor, Niemann Pick disease, type C1, Niemann-Pick C1 protein, NPC, NPC1, NPC1,
Immunogen: Recombinant protein
Conjugate: Unconjugated
Modification: Unmodified