Anti-RP2 Antibody Picoband® Fluoro647 Conjugated

Protein XRP2 is a protein that in humans is encoded by the RP2 gene. It is mapped to Xp11.3. The RP2 locus has been implicated as one cause of X-linked retinitis pigmentosa. The predicted gene product shows homology with human cofactor C, a protein involved in the ultimate step of beta-tubulin folding. Progressive retinal degeneration may therefore be due to the accumulation of incorrectly folded photoreceptor or neuron-specific tubulin isoforms followed by progressive cell death. The RP2 protein is also involved in regulating the function and extension of outer segment of cone photoreceptors in mice.

Ubiquitous. Expressed in the rod and cone photoreceptors, extending from the tips of the outer segment (OS) through the inner segment (IS) and outer nuclear layer (ONL) and into the synaptic terminals of the outer plexiform layer (ONL) . Also detected in the bipolar, horizontal and amacrine cells in the inner nuclear layer (INL), extending to the inner plexiform layer (IPL) and though the ganglion cell layer (GCL) and into the nerve fiber layer (NFL) (at protein level) .

Acts as a GTPase-activating protein (GAP) involved in trafficking between the Golgi and the ciliary membrane. Involved in localization of proteins, such as NPHP3, to the cilium membrane by inducing hydrolysis of GTP ARL3, leading to the release of UNC119 (or UNC119B) . Acts as a GTPase-activating protein (GAP) for tubulin in concert with tubulin-specific chaperone C, but does not enhance tubulin heterodimerization. Acts as guanine nucleotide dissociation inhibitor towards ADP-ribosylation factor-like proteins.

1. Chapple, J. P., Hardcastle, A. J., Grayson, C., Spackman, L. A., Willison, K. R., Cheetham, M. E.Mutations in the N-terminus of the X-linked retinitis pigmentosa protein RP2 interfere with the normal targeting of the protein to the plasma membrane. Hum. Molec. Genet. 9: 1919-1926, 2000. 2. Evans, R. J., Schwarz, N., Nagel-Wolfrum, K., Wolfrum, U., Hardcastle, A. J., Cheetham, M. E.The retinitis pigmentosa protein RP2 links pericentriolar vesicle transport between the Golgi and the primary cilium. Hum. Molec. Genet. 19: 1358-1367, 2010.