E. coli-derived recombinant human Norrin Lys25-Ser133 Accession # Q00604
Specificity
Detects human Norrin in direct ELISAs and Western blots. In direct ELISAs, approximately 50% cross-reactivity with recombinant mouse Norrin is observed.
Source
N/A
Isotype
IgG
Clonality
Polyclonal
Host
Goat
Gene
NDP
Purity Statement
Antigen Affinity-purified
Endotoxin Note
<0.10 EU per 1 μg of the antibody by the LAL method.
Innovator's Reward
Test in a species/application not listed above to receive a full credit towards a future purchase.
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.
Buffer
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.
Preservative
No Preservative
Concentration
LYOPH
Reconstitution Instructions
Reconstitute at 0.2 mg/mL in sterile PBS.
Notes
This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.
Alternate Names for Norrin/NDP Antibody [Unconjugated]
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.
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.
Limitations
This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.
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