Norrin Antibody

Product Specifications

Background

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.

NCBI Gene ID

4693

Swiss Prot

Q00604

Accession Number

NP_000257

Host

Rabbit

Reactivity

Human

Clonality

Polyclonal

Conjugation

Unconjugated

Type

Primary Antibodies

Field of Research

Signal Transduction

Purification

Norrin Antibody is affinity chromatography purified via peptide column.

Positive Control

Cat. No. 1205 - Jurkat Cell Lysate

Concentration

1 mg/mL

Homology

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

Buffer

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

Modification

None

Shipping Conditions

Blue Ice

Storage Conditions

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.

Fragment

IgG

Symbol

NDP

Positive Control 2

Cat. No. 17-005 - Jurkat Cell Slide

NCBI Official Name

Norrie disease (pseudoglioma)

NCBI Organism

Homo sapiens

Background Reference 01

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.

Background Reference 02

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.

Background Reference 03

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.

Background Reference 04

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.