Recombinant Human Norrin Protein, CF

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Product Details

Summary
Reactivity HuSpecies Glossary
Applications Bioactivity
Format
Carrier-Free

Order Details

Recombinant Human Norrin Protein, CF Summary

Details of Functionality
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.
Source
E. coli-derived human Norrin protein
Lys25-Ser133, with an N-terminal Met
Accession #
N-terminal Sequence
Met
Structure / Form
Disulfide-linked oligomer
Protein/Peptide Type
Recombinant Proteins
Gene
NDP
Purity
>97%, by SDS-PAGE under reducing conditions and visualized by silver stain.
Endotoxin Note
<1.0 EU per 1 μg of the protein by the LAL method.

Applications/Dilutions

Theoretical MW
12.6 kDa.
Disclaimer note: The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors.
Publications
Read Publication using
3014-NR in the following applications:

Packaging, Storage & Formulations

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.
Buffer
Lyophilized from a 0.2 μm filtered solution in HCl.
Purity
>97%, by SDS-PAGE under reducing conditions and visualized by silver stain.
Reconstitution Instructions
Reconstitute at 250 μg/mL in sterile 4 mM HCl.

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Alternate Names for Recombinant Human Norrin Protein, CF

  • EVR2
  • exudative vitreoretinopathy 2 (X-linked)
  • FEVR
  • ND
  • NDP
  • Norrie disease (pseudoglioma)
  • Norrie disease protein
  • Norrin
  • X-linked exudative vitreoretinopathy 2 protein

Background

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.

  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.

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3014-NR
Species: Hu
Applications: Bioactivity

Publications for Norrin/NDP (3014-NR)(1)

We have publications tested in 1 confirmed species: Human.

We have publications tested in 1 application: Bioassay.


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Bioinformatics

Gene Symbol NDP
Uniprot