Human Norrin Antibody Summary
Lys25-Ser133
Accession # Q00604
Applications
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.
Reconstitution Calculator
Preparation and Storage
- 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.
- Perez-Vilar, J. and R.L. Hill (1997) J. Biol. Chem. 272:33410.
- Berger, W. et al. (1992) Nat. Genet. 1:199.
- Berger, W. and H.H. Ropers (2001) “The Metabolic and Molecular Bases of Inherited Diseases”, C.R. Scriver, et al. eds. p. 5977.
- Meitinger, T et al. (1993) Nat Genet 5:376.
- Xu, Q. et al. (2004) Cell 116:883.
- Hartzer, M.K. et al. (1999) Brain Res. Bull. 49:355.
- Wang, Y. et al. (2001) J. Neurosci. 21:4761.
Product Datasheets
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
Filter your results:
Filter by:
-
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 -
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 -
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 -
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 -
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 -
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
FAQs
No product specific FAQs exist for this product, however you may
View all Antibody FAQsReviews for Human Norrin Antibody
There are currently no reviews for this product. Be the first to review Human Norrin Antibody and earn rewards!
Have you used Human Norrin Antibody?
Submit a review and receive an Amazon gift card.
$25/€18/£15/$25CAN/¥75 Yuan/¥2500 Yen for a review with an image
$10/€7/£6/$10 CAD/¥70 Yuan/¥1110 Yen for a review without an image