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HPA000835

Sigma-Aldrich

Anti-NPC2 antibody produced in rabbit

enhanced validation

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

同義詞:

Anti-Epididymal secretory protein E1 precursor antibody produced in rabbit, Anti-Niemann-Pick disease type C2 protein antibody produced in rabbit, Anti-hE1 antibody produced in rabbit

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About This Item

分類程式碼代碼:
12352203
人類蛋白質圖譜編號:
NACRES:
NA.41

生物源

rabbit

共軛

unconjugated

抗體表格

affinity isolated antibody

抗體產品種類

primary antibodies

無性繁殖

polyclonal

產品線

Prestige Antibodies® Powered by Atlas Antibodies

形狀

buffered aqueous glycerol solution

物種活性

human, rat, mouse

加強驗證

orthogonal RNAseq
Learn more about Antibody Enhanced Validation

技術

immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:1000-1:2500

免疫原序列

PVQFKDCGSVDGVIKEVNVSPCPTQPCQLSKGQSYSVNVTFTSNIQSKSSKAVVHGILMGVPVPFPIPEPDGCKSGINCPIQKDKTYSYLNKLPVKSEYPSIKLVVEWQLQDDKNQSLFCWEIPVQIVSH

UniProt登錄號

運輸包裝

wet ice

儲存溫度

−20°C

目標翻譯後修改

unmodified

基因資訊

human ... NPC2(10577)

一般說明

Niemann Pick type C (NPC2) protein is a member of the Niemann Pick type C family of proteins.
The gene NPC2 (Niemann-Pick disease type C2) is mapped to human chromosome 14q24.3. It contains only five exons spanning a length of 13.5kb and encodes a soluble lysosomal protein of 132 amino acids.

免疫原

Epididymal secretory protein E1 precursor recombinant protein epitope signature tag (PrEST)

應用

All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.

The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
Anti-NPC2 antibody produced in rabbit has been used in western blotting and immunohistochemistry.

生化/生理作用

Niemann-Pick type C is a lysosomal storage disease (fatal autosomal recessive disorder) caused due to mutations in NPC1 and NPC2 genes. It is an inherited disorder in which cholesterol and other lipids accumulate in the late endosomal/lysosomal compartment. The disease is characterized by hepatosplenomegaly and progressive neurological deterioration. NPC2 protein is one of the most abundant components of the epididymal fluid and contains a functional cholesterol-binding site that can transfer cholesterol between membranes. It has a key role for cellular cholesterol regulation in the innate immune response. The protein can be involved in regulation of cholesterol levels in spermatozoa during epididymal maturation. NPC2 acts as a specific regulator of arachidonic acid (AA) metabolism and inflammation and may help in the treatment of inflammatory diseases characterized by the presence of activated fibroblasts.

特點和優勢

Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.

Every Prestige Antibody is tested in the following ways:
  • IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
  • Protein array of 364 human recombinant protein fragments.

聯結

Corresponding Antigen APREST70511

外觀

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide

法律資訊

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

免責聲明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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儲存類別代碼

10 - Combustible liquids

水污染物質分類(WGK)

WGK 1

閃點(°F)

Not applicable

閃點(°C)

Not applicable

個人防護裝備

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)


分析證明 (COA)

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Milan Hodošček et al.
International journal of molecular sciences, 19(9) (2018-09-06)
The Niemann Pick type C (NPC) proteins, NPC1 and NPC2, are involved in the lysosomal storage disease, NPC disease. The formation of a NPC1⁻NPC2 protein⁻protein complex is believed to be necessary for the transfer of cholesterol and lipids out of
Matthew Wheelwright et al.
Journal of immunology (Baltimore, Md. : 1950), 192(5), 2280-2290 (2014-02-07)
A role for vitamin A in host defense against Mycobacterium tuberculosis has been suggested through epidemiological and in vitro studies; however, the mechanism is unclear. In this study, we demonstrate that vitamin A-triggered antimicrobial activity against M. tuberculosis requires expression
Afagh Alavi et al.
Molecular genetics and metabolism, 110(1-2), 139-144 (2013-06-25)
We report identification of a homozygous mutation in NPC2 in two Iranian siblings with a neurologic dysfunction whose disease had not been diagnosed prior to our genetic analysis. The mutation was identified by exome sequencing. The finding resulted in diagnosis
Heiko Runz et al.
Human mutation, 29(3), 345-350 (2007-12-18)
Niemann-Pick type C (NPC) disease is a rare autosomal-recessive lysosomal storage disease typically accompanied by progressive impairment of nervous system and liver function. Biochemically, the disorder presents with an inhibited egress of cholesterol and glycosphingolipids from endosomal and lysosomal compartments
Blair R Roszell et al.
PloS one, 8(7), e67084-e67084 (2013-07-12)
Niemann-Pick C (NPC) disease is due to loss of NPC1 or NPC2 protein function that is required for unesterified cholesterol transport from the endosomal/lysosomal compartment. Though lung involvement is a recognized characteristic of Niemann-Pick type C disease, the pathological features

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