Anti-PDE6 beta/PDE6B Antibody Picoband® Fluoro647 Conjugated

Photon absorption triggers a signaling cascade in rod photoreceptors that activates cGMP phosphodiesterase (PDE), resulting in the rapid hydrolysis of cGMP, closure of cGMP-gated cation channels, and hyperpolarization of the cell. PDE is a peripheral membrane heterotrimeric enzyme made up of alpha, beta, and gamma subunits. This gene encodes the beta subunit. Mutations in this gene result in retinitis pigmentosa and autosomal dominant congenital stationary night blindness. Multiple transcript variants encoding different isoforms have been found for this gene.

Expressed in fibroblasts, heart, skeletal muscle, brain and pancreas. Expressed at an intermediate level in lung and kidney, and at a low level in liver and placenta. Expressed at a lower level in fibroblasts from hypertrophic scar lesional skin and in fibroblasts from patients with systemic sclerosis than in normal skin fibroblasts.

Seems to mediate adhesion by cell surface integrin binding. May serve as a communication link between the dermal fibroblast cell surface and its extracellular matrix environment. Enhances TGFB1 activity. Inhibits cell proliferation. Accelerates collagen fibril formation, and stabilizes collagen fibrils against low-temperature dissociation (By similarity) .

1. Hmani-Aifa, M., Benzina, Z., Zulfiqar, F., Dhouib, H., Shahzadi, A., Ghorbel, A., Rebai, A., Soderkvist, P., Riazuddin, S., Kimberling, W. J., Ayadi, H. Identification of two new mutations in the GPR98 and the PDE6B genes segregating in a Tunisian family. Europ. J. Hum. Genet. 17: 474-482, 2009. 2. Tsang, S. H., Woodruff, M. L., Jun, L., Mahajan, V., Yamashita, C. K., Pedersen, R., Lin, C.-S., Goff, S. P., Rosenberg, T., Larsen, M., Farber, D. B., Nusinowitz, S. Transgenic mice carrying the H258N mutation in the gene encoding the beta-subunit of phosphodiesterase-6 (PDE6B) provide a model for human congenital stationary night blindness. Hum. Mutat. 28: 243-254, 2007. 3. Zhao, L., Zabel, M. K., Wang, X., Ma, W., Shah, P., Fariss, R. N., Qian, H., Parkhurst, C. N., Gan, W.-B., Wong, W. T. Microglial phagocytosis of living photoreceptors contributes to inherited retinal degeneration. EMBO Molec. Med. 7: 1179-1197, 2015.