Human Norrin Antibody

Catalog # Availability Size / Price Qty
AF3014
AF3014-SP
Product Details
Citations (6)
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Human Norrin Antibody Summary

Species Reactivity
Human
Specificity
Detects human Norrin in direct ELISAs and Western blots. In direct ELISAs, approximately 50% cross-reactivity with recombinant mouse Norrin is observed.
Source
Polyclonal Goat IgG
Purification
Antigen Affinity-purified
Immunogen
E. coli-derived recombinant human Norrin
Lys25-Ser133
Accession # Q00604
Formulation
Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied either lyophilized or as a 0.2 µm filtered solution in PBS.
Endotoxin Level
<0.10 EU per 1 μg of the antibody by the LAL method.
Label
Unconjugated

Applications

Recommended Concentration
Sample
Western Blot
0.1 µg/mL
Recombinant Human Norrin (Catalog # 3014-NR)
Immunohistochemistry
5-15 µg/mL
Immersion fixed paraffin-embedded sections of human brain (cerebellum and cortex) subjected to Antigen Retrieval Reagent-Basic (Catalog # CTS013)
Blockade of Receptor-ligand Interaction
In a functional ELISA, 2-6 µg/mL of this antibody will block 50% of the binding of 0.25 μg/mL of Recombinant Mouse Frizzled-4 Fc Chimera to immobilized Recombinant Human Norrin coated at 0.5 µg/mL (100 µL/well). At 50 μg/mL, this antibody will block >80% of the binding.
 

Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.

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Preparation and Storage

Reconstitution
Reconstitute at 0.2 mg/mL in sterile PBS.
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Shipping
Lyophilized product is shipped at ambient temperature. Liquid small pack size (-SP) is shipped with polar packs. Upon receipt, store immediately at the temperature recommended below.
Stability & Storage
Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
  • 12 months from date of receipt, -20 to -70 °C as supplied.
  • 1 month, 2 to 8 °C under sterile conditions after reconstitution.
  • 6 months, -20 to -70 °C under sterile conditions after reconstitution.

Background: Norrin

Norrin (also called Norrie Disease Protein or NDP) is a secreted regulatory protein that remains tightly associated with the extracellular matrix (1). At least 70 mutations of Norrin have been identified in Norrie disease (X-linked retinal dysplasia) or in a minority of X-linked familial exudative vitreoretinopathy (FEVR). Both are disorders of retinal vascularization that cause blindness (2, 3). Norrin consists of disulfide-linked homodimers that oligomerize further via disulfide bridges to form higher order oligomers containing up to ten units of 12 kDa each. The cysteine-rich C-terminal domain of Norrin is homologous to von Willebrand factor, several extracellular mucin proteins, and members of the TGF-beta family. Molecular modeling studies predict that Norrin assumes a cysteine-knot structure typical for the TGF‑ beta family (4). Although Norrin is not related to Wnt family proteins, it functions like a Wnt protein in that it binds with high affinity to the receptor Frizzled-4, requires LDL receptor-related protein (LRP) as a co-receptor, and induces activation of the canonical Wnt signaling pathway (5). Norrin and Frizzled-4 are expressed at relatively low levels in tissues displaying vascular phenotypes and genetic disruption of either gene in mice gives phenotypes with marked similarities (5, 6, 7). Human Norrin shares 100%, 96%, 95%, 94% and 90% amino acid identity with rhesus macaque, canine, bovine, mouse, and chick Norrin, respectively.

References
  1. Perez-Vilar, J. and R.L. Hill (1997) J. Biol. Chem. 272:33410.
  2. Berger, W. et al. (1992) Nat. Genet. 1:199.
  3. Berger, W. and H.H. Ropers (2001) “The Metabolic and Molecular Bases of Inherited Diseases”, C.R. Scriver, et al. eds. p. 5977.
  4. Meitinger, T et al. (1993) Nat Genet 5:376.
  5. Xu, Q. et al. (2004) Cell 116:883.
  6. Hartzer, M.K. et al. (1999) Brain Res. Bull. 49:355.
  7. Wang, Y. et al. (2001) J. Neurosci. 21:4761.
Entrez Gene IDs
4693 (Human)
Alternate Names
EVR2; exudative vitreoretinopathy 2 (X-linked); FEVR; ND; NDP; Norrie disease (pseudoglioma); Norrie disease protein; Norrin; X-linked exudative vitreoretinopathy 2 protein

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Citations for Human Norrin Antibody

R&D Systems personnel manually curate a database that contains references using R&D Systems products. The data collected includes not only links to publications in PubMed, but also provides information about sample types, species, and experimental conditions.

6 Citations: Showing 1 - 6
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  1. Investigating the Impact of Dimer Interface Mutations on Norrin's Secretion and Norrin/?-Catenin Pathway Activation
    Authors: Liu, M;Dai, E;Yang, M;Li, S;Fan, L;Liu, Y;Xiao, H;Zhao, P;Yang, Z;
    Investigative ophthalmology & visual science
    Species: Human
    Sample Types: Cell Lysates
    Applications: Western Blot
  2. Systemic gene therapy rescues retinal dysfunction and hearing loss in a model of Norrie disease
    Authors: Pauzuolyte, V;Patel, A;Wawrzynski, JR;Ingham, NJ;Leong, YC;Karda, R;Bitner-Glindzicz, M;Berger, W;Waddington, SN;Steel, KP;Sowden, JC;
    EMBO molecular medicine
    Species: Human
    Sample Types: Cell Lysates
    Applications: Western Blot
  3. Norrin mediates tumor-promoting and -suppressive effects in glioblastoma via Notch and WNT
    Authors: A El-Sehemy, HJ Selvadurai, A Ortin-Mart, NT Pokrajac, Y Mamatjan, N Tachibana, KJ Rowland, L Lee, NI Park, KD Aldape, P Dirks, VA Wallace
    J. Clin. Invest., 2020-06-01;0(0):.
    Species: Human
    Sample Types: Cell Culture Lysates
    Applications: Western Blot
  4. A novel signaling pathway regulates colon cancer angiogenesis through Norrin.
    Authors: Planutis, Kestutis, Planutiene, Marina, Holcombe, Randall
    Sci Rep, 2014-07-09;4(0):5630.
    Species: Human
    Sample Types: Whole Cells
    Applications: Neutralization
  5. Norrin mediates neuroprotective effects on retinal ganglion cells via activation of the Wnt/beta-catenin signaling pathway and the induction of neuroprotective growth factors in Muller cells.
    Authors: Seitz R, Hackl S, Seibuchner T, Tamm ER, Ohlmann A
    J. Neurosci., 2010-04-28;30(17):5998-6010.
    Species: Human
    Sample Types: Cell Lysates
    Applications: Western Blot
  6. Norrin promotes vascular regrowth after oxygen-induced retinal vessel loss and suppresses retinopathy in mice.
    Authors: Ohlmann A, Seitz R, Braunger B, Seitz D, Bosl MR, Tamm ER
    J. Neurosci., 2010-01-06;30(1):183-93.
    Species: Human
    Sample Types: Cell Culture Supernates
    Applications: Western Blot

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