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Anti-Niemann Pick C1/NPC1 Antibody (1B891) is a Rabbit antibody targeting Niemann Pick C1/NPC1. Anti-Niemann Pick C1/NPC1 Antibody (1B891) can be used in WB,ICC,IF,IHC,FCM.
Pack Size | Price | Availability | Quantity |
---|---|---|---|
50 μL | $298 | 7-10 days | |
100 μL | $496 | 7-10 days |
Description | Anti-Niemann Pick C1/NPC1 Antibody (1B891) is a Rabbit antibody targeting Niemann Pick C1/NPC1. Anti-Niemann Pick C1/NPC1 Antibody (1B891) can be used in WB,ICC,IF,IHC,FCM. |
Ig Type | IgG |
Reactivity | Human,Mouse,Rat |
Application | |
Recommended Dose | WB: 1:500-1000; IHC: 1:50-200; ICC:IF: 1:50-200; FCM: 1:50-100 |
Antibody Type | Monoclonal |
Host Species | Rabbit |
Construction | Recombinant Antibody |
Purification | ProA affinity purified |
Appearance | Liquid |
Formulation | 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide. |
Research 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. |
Conjucates | Unconjugated |
Immunogen | Recombinant Protein |
Antigen Species | Recombinant Protein |
Uniprot ID |
Molecular Weight | Theoretical: 142 kDa. |
Stability & Storage | Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. |
Transport | Shipping with blue ice. |
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