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Recombinant Human Norrin Protein, CF

Catalog # 3014-NR | R&D Systems, Inc. a Bio-Techne Brand
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3014-NR-025

Key Product Details

Source

E. coli

Accession #

Structure / Form

Disulfide-linked oligomer

Conjugate

Unconjugated

Applications

Bioactivity

Product Specifications

Source

E. coli-derived human Norrin protein
Lys25-Ser133, with an N-terminal Met

Purity

>97%, by SDS-PAGE under reducing conditions and visualized by silver stain.

Endotoxin Level

<1.0 EU per 1 μg of the protein by the LAL method.

N-terminal Sequence Analysis

Met

Predicted Molecular Mass

12.6 kDa

Activity

Measured by its ability to activate Wnt induced TCF reporter activity in HEK293 human embryonic kidney cells expressing human Frizzled-4 and human LRP-5.
The ED50 for this effect is 30-150 ng/ml.

Formulation, Preparation and Storage

3014-NR
Formulation Lyophilized from a 0.2 μm filtered solution in HCl.
Reconstitution Reconstitute at 250 μg/mL in sterile 4 mM HCl.
Shipping The product is shipped at ambient temperature. Upon receipt, store it 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.
  • 3 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.

Alternate Names

NDP

Entrez Gene IDs

4693 (Human)

Gene Symbol

NDP

UniProt

Product Documents for Recombinant Human Norrin Protein, CF

Certificate of Analysis

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Note: Certificate of Analysis not available for kit components.

Product Specific Notices for Recombinant Human Norrin Protein, CF

For research use only

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