NPC1 Recombinant monoclonal antibody

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• NPC1 Recombinant monoclonal antibody (86233-5-RR)

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  WB analysis using 86233-5-RR

  100℃ incubated and 37℃ incubated HEK-293 cell lysates were subjected to SDS PAGE followed by western blot with 86233-5-RR (NPC1 antibody) at dilution of 1:5000 incubated at room temperature for 1.5 hours.

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  IF Staining of HepG2 using 86233-5-RR

  Immunofluorescent analysis of (-20°C Methanol) fixed HepG2 cells using NPC1 antibody (86233-5-RR, Clone: 250854D2 ) at dilution of 1:400 and CoraLite®488-Conjugated Goat Anti-Rabbit IgG(H+L) (SA00013-2).

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  Affinity and Kinetic Characterization of 86233-5-RR

  Biolayer interferometry (BLl) kinetic assays of 86233-5-RR against Human NPC1 were performed. The affinity constant is 12.3 pM.

NPC1 Uni-rAb^® Recombinant Antibody for WB, IF/ICC, ELISA

Cat No. 86233-5-RR

Clone No.250854D2

Host / Isotype

Rabbit / IgG

Reactivity

human

Applications

WB, IF/ICC, ELISA

Synonyms

Niemann Pick C1 protein, Niemann Pick disease, type C1, Niemann-Pick C1 protein, NPC intracellular cholesterol transporter 1

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Tested Applications

• Positive WB detected in: 37°C incubated HEK-293 cells
• Positive IF/ICC detected in: HepG2 cells

Recommended dilution

• Western Blot (WB): WB : 1:2000-1:10000
• Immunofluorescence (IF)/ICC: IF/ICC : 1:200-1:800

It is recommended that this reagent should be titrated in each testing system to obtain optimal results.

Sample-dependent, Check data in validation data gallery.

Product Information

86233-5-RR targets NPC1 in WB, IF/ICC, ELISA applications and shows reactivity with human samples.

• Tested Reactivity: human
• Host / Isotype: Rabbit / IgG
• Class: Recombinant
• Type: Antibody
• Immunogen:

  CatNo: Ag4946

  Product name: Recombinant human NPC1 protein

  Source: e coli.-derived, PGEX-4T

  Tag: GST

  Domain: 23-266 aa of BC063302

  Sequence: QSCVWYGECGIAYGDKRYNCEYSGPPKPLPKDGYDLVQELCPGFFFGNVSLCCDVRQLQTLKDNLQLPLQFLSRCPSCFYNLLNLFCELTCSPRQSQFLNVTATEDYVDPVTNQTKTNVKELQYYVGQGFANAMYNACRDVEAPSSNDKALGLLCGKDADACNATNWIEYMFNKDNGQAPFTITPVFSDFPVHGMEPMNNATKGCDESVDEVTAPCSCQDCSIVCGPKPQPPPPPAPWTILGLD

• Full Name: Niemann-Pick disease, type C1
• Calculated Molecular Weight: 142 kDa
• Observed Molecular Weight: 160-200 kDa
• GenBank Accession Number: BC063302
• Gene Symbol: NPC1
• Gene ID (NCBI): 4864
• RRID: AB_3744734
• Conjugate: Unconjugated
• Form: Liquid
• Purification Method: Protein A purification
• UNIPROT ID: O15118
• Storage Buffer: PBS with 0.02% sodium azide and 50% glycerol, pH 7.3.
• Storage Conditions: Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20^oC storage. 20ul sizes contain 0.1% BSA.

Background Information

Niemann-Pick Type C (NPC) disease is a lysosomal storage disorder characterized by the 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.