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  • KD/KO Validated

NPC1 Polyclonal antibody

NPC1 Polyclonal Antibody for WB, IF/ICC, IHC, ELISA

Host / Isotype

Rabbit / IgG

Reactivity

human, mouse and More (1)

Applications

WB, IF/ICC, IHC, ELISA

Conjugate

Unconjugated

Cat No : 13926-1-AP

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Synonyms

Niemann Pick C1 protein, Niemann Pick disease, type C1, NPC1



经过测试的应用

Positive WB detected inunboiled HEK-293 cells, unboiled mouse brain tissue, HepG2 cells, HeLa cells
Positive IHC detected inhuman liver cancer tissue, human placenta tissue, human brain tissue
Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0
Positive IF detected inNeuro-2a cells, HepG2 cells

推荐稀释比

ApplicationDilution
Western Blot (WB)WB : 1:500-1:2000
Immunohistochemistry (IHC)IHC : 1:50-1:500
Immunofluorescence (IF)IF : 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.

产品信息

13926-1-AP targets NPC1 in WB, IF, IHC, ELISA applications and shows reactivity with human, mouse samples.

Tested Applications WB, IF/ICC, IHC, ELISA Application Description
Cited ApplicationsWB, IHC
Tested Reactivity human, mouse
Cited Reactivityhuman, mouse, pig
Immunogen NPC1 fusion protein Ag4946 种属同源性预测
Host / Isotype Rabbit / IgG
Class Polyclonal
Type Antibody
Full Name Niemann-Pick disease, type C1
Synonyms Niemann Pick C1 protein, Niemann Pick disease, type C1, NPC1
Calculated Molecular Weight 142 kDa
Observed Molecular Weight 160-200 kDa
GenBank Accession NumberBC063302
Gene Symbol NPC1
Gene ID (NCBI) 4864
RRIDAB_2152050
Conjugate Unconjugated
Form Liquid
Purification MethodAntigen affinity purification
UNIPROT IDO15118
Storage Buffer PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
Storage ConditionsStore at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage.

背景介绍

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.

实验方案

Product Specific Protocols
WB protocol for NPC1 antibody 13926-1-APDownload protocol
IHC protocol for NPC1 antibody 13926-1-APDownload protocol
IF protocol for NPC1 antibody 13926-1-APDownload protocol
Standard Protocols
Click here to view our Standard Protocols

发表文章

SpeciesApplicationTitle
humanWB

Cell

Cholesterol Transport through Lysosome-Peroxisome Membrane Contacts.

Authors - Bei-Bei Chu
  • KD Validated
humanWB

Nat Commun

A genome-wide CRISPR screen identifies host factors that regulate SARS-CoV-2 entry.

Authors - Yunkai Zhu
humanWB

EMBO J

Insufficiency of ciliary cholesterol in hereditary Zellweger syndrome.

Authors - Tatsuo Miyamoto
  • KO Validated
humanWB

Proc Natl Acad Sci U S A

Cholesterol trafficking is required for mTOR activation in endothelial cells.

Authors - Xu Jing J
  • KD Validated
mouseWB

Proc Natl Acad Sci U S A

Inhibition of sphingolipid synthesis improves outcomes and survival in GARP mutant wobbler mice, a model of motor neuron degeneration.

Authors - Constance S Petit
mouseWB

Cell Rep

The GARP Complex Is Involved in Intracellular Cholesterol Transport via Targeting NPC2 to Lysosomes.

Authors - Jian Wei