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Niemann Pick C2 Antibody (YA1399)

(Synonyms: HE1; EDDM1)
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Niemann Pick C2 Antibody (YA1399) is a Rabbit-derived and non-conjugated IgG monoclonal antibody, targeting to Niemann Pick C2.

For research use only. We do not sell to patients.
  • Host:

    Rabbit

  • Isotype:

    IgG

  • Application:

    WB, IHC-P

  • Reactivity :

    Human, Mouse, Rat

  • Formulation:

    Supplied in 50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40% Glycerol, 0.01% Sodium azide and 0.05% BSA

  • Conjugation:
    Non-conjugated
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To place orders, for customer services and technical support, please contact: MedChemExpress USA

Tel: 609-228-6898 E-mail: [email protected] [email protected]

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Applications

Application
WB Info
WB: Western Blot
IHC-P Info
IHC-P: Immunohistochemistry-Paraffin
Dilution Ratio 1:500-1:1000 1:500-1:2000

Product Details

Description

Niemann Pick C2 Antibody (YA1399) is a Rabbit-derived and non-conjugated IgG monoclonal antibody, targeting to Niemann Pick C2.

  • Host Rabbit
  • Clonality Recombinant,Monoclonal
  • Species Reactivity
    Human, Mouse, Rat
  • Observed Molecular Weight
    Observed band size: 16-18 kDa Info
    Note: Due to possible protein modifications or aggregation, the molecular weight should be confirmed by actual measurement, and the predicted value is for reference only.
  • Calculated Molecular Weight Predicted band size: 17 kDa
Species Reactivity Database
Immunogen

Recombinant protein of human Niemann Pick C2 aa20-151.

Sensitivity

Endogenous

Purification

Affinity Purified

Conjugation

Non-conjugated

Modification

Unmodified

Isotype

IgG

Product Properties

  • Appearance

    Solution

  • Formulation

    Supplied in 50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40% Glycerol, 0.01% Sodium azide and 0.05% BSA

  • Concentration

    Batch-dependent, Please check the COA for the concentration of each lot. Check Lot Concentration

  • Storage & Stability

    Stored at -20°C for 1 year. Avoid repeated freeze / thaw cycles.

  • Shipping

    Shipping with blue ice.

Background

  • Function

    Niemann Pick C2 is an Intracellular cholesterol transporter which acts in concert with NPC1 and plays an important role in the egress of cholesterol from the lysosomal compartment. Unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes is transferred by NPC2 to the cholesterol-binding pocket in the N-terminal domain of NPC1. May bind and mobilize cholesterol that is associated with membranes. NPC2 binds cholesterol with a 1:1 stoichiometry. Can bind a variety of sterols, including lathosterol, desmosterol and the plant sterols stigmasterol and beta-sitosterol. The secreted form of NCP2 regulates biliary cholesterol secretion via stimulation of ABCG5/ABCG8-mediated cholesterol transport[1][2][3][4][5][6][7].

  • Subcellular Localization

    Secreted; Endoplasmic reticulum; Lysosome

  • Expression


    Tissue_specificity:Detected in gallbladder bile (PubMed:21315718) . Detected in fibroblasts, kidney, liver, spleen, small intestine, placenta and testis (at protein level) (PubMed:11125141) . Epididymis

    Induction:Down-regulated in response to enterovirus 71 (EV71) infection

  • Isoforms & Post-Translational Modification

    P61916 has 2 isomers: P61916-1: 16570 Da (predicted); P61916-2: 13416 Da (predicted).

  • Subunit

    Interacts with NPC1 (via the second lumenal domain) in a cholestrol-dependent manner (PubMed:18772377, PubMed:27238017, PubMed:27551080).

  • SwissProt ID

    P61916

  • Gene ID
    10577 [NCBI]
  • Synonyms

    HE1; EDDM1

  • Research Field

    Cardiovascular

References

[1]. Naureckiene S, et al. Identification of HE1 as the second gene of Niemann-Pick C disease. Science. 2000 Dec 22;290(5500):2298-301. [Content Brief]

[2]. Chikh K, et al. Niemann-Pick type C disease: subcellular location and functional characterization of NPC2 proteins with naturally occurring missense mutations. Hum Mutat. 2005 Jul;26(1):20-8. [Content Brief]

[3]. Liou HL, et al. NPC2, the protein deficient in Niemann-Pick C2 disease, consists of multiple glycoforms that bind a variety of sterols. J Biol Chem. 2006 Dec 1;281(48):36710-23. [Content Brief]

[4]. Infante RE, et al. NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomes. Proc Natl Acad Sci U S A. 2008 Oct 7;105(40):15287-92. [Content Brief]

[5]. Berzina Z, et al. Niemann-Pick C2 protein regulates sterol transport between plasma membrane and late endosomes in human fibroblasts. Chem Phys Lipids. 2018 Jul;213:48-61. [Content Brief]

[6]. Gong X, et al. Structural Insights into the Niemann-Pick C1 (NPC1)-Mediated Cholesterol Transfer and Ebola Infection. Cell. 2016 Jun 2;165(6):1467-1478. [Content Brief]

[7]. Xu Z, et al. Regulation of sterol transport between membranes and NPC2. Biochemistry. 2008 Oct 21;47(42):11134-43. [Content Brief]

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Niemann Pick C2 Antibody (YA1399) Related Classifications

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100 mg

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Measured by its ability to inhibit anti-CD3-induced proliferation of stimulated CTLL-2 mouse cytotoxic T cells. The ED50 this effect is 0.8023 μg/ml, corresponding to a specific activity is 1246.42 units/mg.

Assay Types
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