Product Datasheet  
Niemann Pick C1 Antibody  
Catalog Number: 49838  
Technical:tech@swbio.com  
Information:info@swbio.com  
Description  
  • host_species：  
  • Rabbit
  • Amount：  
  • 100μgμg
  • Swiss-Prot No.：  
  • Swiss-Prot#:O15118
  • Form of Antibody：  
  • 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.
  • Storage：  
  • Store at -20˚C
  • Immunogen：  
  • Recombinant protein
  • reactivity：  
  • Hu, Ms, Rt
  • appl_detail：  
  • WB: 1:500-1:1,000
    IHC: 1:50-1:200
    ICC/IF: 1:50-1:200

    FC: 1:50-1:100
  • other_names：  
  • Niemann Pick C1 protein precursor antibody
    Niemann Pick disease, type C1 antibody
    Niemann-Pick C1 protein antibody
    NPC antibody
    NPC1 antibody
    NPC1_HUMAN antibody
  • Purification：  
  • ProA affinity purified
  • Specificity：  
  • Applications：  
  • WB,ICC,IF,IHC,FC
  • 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.



 
© Signalway Biotechnology All Rights Reserved.