Norrin Antilichaam

Norrin Antibody: Norrie disease is an X-linked genetic disorder characterized by progressive atrophy of the eyes, mental disturbances and deafness. The gene responsible for this disease was initially identified through positional cloning. Norrin, the gene product, encodes a small secreted, cysteine-rich protein that is thought to act as a ligand for the Wnt-receptor/beta-catenin signal pathway despite having sequence homology with the Wnt family of proteins. Mice lacking this gene have abnormal blood vessel growth in the vitreous and a disorganized retina; transgenic ectopic expression of Norrin restores normal retinal vasculature. Recent evidence shows that Norrin can attenuate tPA and uPA-mediated death of transformed rat retinal ganglion cells (RGC-5) by activating the Wnt/beta-catenin pathway and regulating the phosphorylation of LRP-1, a cell surface receptor for tPA and uPA, suggesting the Norrin may function in vivo by regulating kinases which may alter the phosphorylation of LRP-1.

4693

Q00604

NP_000257

Human

Unconjugated

Primary Antibodies

Signal Transduction

Norrin Antibody is affinity chromatography purified via peptide column.

Cat. No. 1205 - Jurkat Cell Lysate

1 mg/mL

Predicted species reactivity based on immunogen sequence: Bovine: (83%), Mouse: (93%)

Norrin Antibody is supplied in PBS containing 0.02% sodium azide.

None

Blue Ice

Norrin antibody can be stored at 4˚ C for three months and -20˚ C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

IgG

NDP

Cat. No. 17-005 - Jurkat Cell Slide

Norrie disease (pseudoglioma)

Homo sapiens

Bergen W, Meindl A, van de Pol TJ, et al. Isolation of a candidate gene for Norrie disease by positional cloning. Nat. Genet.1992; 1:199-203.

Meitinger T, Meindl A, Bork P, et al. Molecular modelling of the Norrie disease protein predicts a cysteine knot growth factor tertiary structure. Nat. Genet.1993; 5:376-80.

Xu Q, Wang Y, Dabdoub A, et al. Vascular development in the retina and inner ear: control by Norrin and Frizzled-4, a high-affinity ligand-receptor pair. Cell2004; 116:883-95.

Ohlmann A, Scholz M, Goldwich A, et al. Ectopic norrin induces growth of ocular capillaries and restores normal retinal angiogenesis. J. Neurosci.2005; 25:1701-10.

Norrin Antibody: ND, EVR2, FEVR, Norrin, Norrie disease protein

ELISA, WB, ICC

4557789

Liquid