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/b-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/b-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.
Applications:Suitable for use in ELISA, Western Blot and Immunocytochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1-2ug/mlOptimal dilutions to be determined by the researcher.
Positive Control:Jurkat Cell Lysate
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for at least 12 months.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ug
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, 0.02% sodium azide.
Specificity：Recognizes human Norrin.
Isotype：IgG
Calc Applications Abbrev：E IC WB
Calc Crossreactivity：Hu
Immunogen：Synthetic peptide corresponding to 18aa from near the N-terminus of human Norrin.
