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Product Name
NPC1 antibody
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Description
NPC1 Rabbit Polyclonal antibody. Positive IHC detected in human brain tissue, human placenta tissue. Positive IF detected in HepG2 cells. Positive WB detected in HEK-293 cells, HepG2 cells. Observed molecular weight by Western-blot: 160 kDa,170 kDa
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Tested applications
ELISA, WB, IHC, IF
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Species reactivity
Human; other species not tested.
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Alternative names
Niemann Pick C1 protein antibody; Niemann Pick disease antibody; type C1 antibody; NPC1 antibody
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Isotype
Rabbit IgG
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Preparation
This antibody was obtained by immunization of NPC1 recombinant protein (Accession Number: NM_000271). Purification method: Antigen affinity purified.
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Clonality
Polyclonal
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Formulation
PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
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Storage instructions
Store at -20℃. DO NOT ALIQUOT
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Applications
Recommended Dilution:
WB: 1:500-1:5000
IHC: 1:20-1:200
IF: 1:50-1:500
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Validations
HEK-293 cells were subjected to SDS PAGE followed by western blot with Catalog No:113209(NPC1 antibody) at dilution of 1:1000
Immunohistochemistry of paraffin-embedded human brain tissue slide using Catalog No:113209(NPC1 Antibody) at dilution of 1:50 (under 10x lens)
Immunohistochemistry of paraffin-embedded human brain tissue slide using Catalog No:113209(NPC1 Antibody) at dilution of 1:50 (under 40x lens)
Immunofluorescent analysis of (10% Formaldehyde) fixed HepG2 cells using Catalog No:113209(NPC1 Antibody) at dilution of 1:50 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
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Background
Niemann-Pick Type C (NPC) disease is a lysosomal storage disorder characterized by accumulation of unesterified cholesterol and other lipids in the endolysosomal system. NPC disease results from a defect in either of two distinct cholesterol-binding proteins: a transmembrane protein, NPC1, and a small soluble protein, NPC2. NPC1 or NPC2 deficiency models showed that the functions of these two proteins within lysosomes are linked closely. NPC1 is a typical transmembrane protein and contains a number of modification sites for glycosylation. Defects in NPC1 are the cause of Niemann-Pick disease type C1 which exhibits highly variable clinical phenotype. Moreover, NPC1 may play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals.
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References
- Xu J, Dang Y, Ren YR, Liu JO. Cholesterol trafficking is required for mTOR activation in endothelial cells. Proceedings of the National Academy of Sciences of the United States of America. 107(10):4764-9. 2010.
- Chu BB, Liao YC, Qi W. Cholesterol transport through lysosome-peroxisome membrane contacts. Cell. 161(2):291-306. 2015.
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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"