We establish animal models of retina disease, mostly transgenics and knockouts, to investigate mechanisms leading to retina dystrophies (retinitis pigmentosa, cone dystrophies, Leber congenital amaurosis, macular degneration). The focus is on components involved in phototransduction, recovery from photobleaching, and retinol recycling through the retinal pigment epithelium. We are further interested in membrane protein transport from the endoplasmic reticulum to the photosensitive cilia in rods and cones, particularly in lipid binding proteins, small GTPases, and molecular motors that transport cargo to the outer segments.
- Mammalian phototransduction and animal models
- The visual Cycle and retina disease
- Membrane protein transport in photoreceptors
- Howes K, Pennesi ME, Sokal I, Church-Kopish J, Schmidt B, Margolis P, Frederick JM, Chen J, Rieke FM, Palczewski K, Wu SM, Detwiler P, Baehr W (2002). GCAP1 rescues rod photoreceptor response in GCAP1/2 knockout mice. EMBO J. 21, 1545-54.
- Zhang H, Liu X-H, Zhang K, Chen C-K, Frederick JM, Prestwich GD, and Baehr W (2004). Photoreceptor cGMP Phosphodiesterase δ-Subunit (PDEδ) Functions as a Prenyl Binding Protein. J. Biol. Chem. 279, 407-13. Epub 2003 Oct 15. Citations 01100507
- Baehr W, Karan S, Maeda T, Luo DG, Li S, Bronson JD, Watt CB, Yau KW, Frederick JM, Palczewski K (2007). The function of Guanylate Cyclase 1 (GC1) and Guanylate Cyclase 2 (GC2) in rod and cone photoreceptors. J. Biol. Chem., 2007;282 8837-8847. Epub 2007, Jan 26.
- Zhang H, Li S, Detwiler P, Rieke F, Frederick J, and Baehr W (2007). Deletion of PrBP/δ impedes transport of GRK1 and PDE to photoreceptor outer segments. Proc Natl Acad Sci USA, 104(21):8857-62.
- Zhang H, Fan J, Li S, Karan S, Rohrer B, Palczewski K, Frederick JM, Crouch RK and Baehr W (2008). Trafficking of Membrane-associated Proteins to Cone Photoreceptor Outer Segments Requires 11-cis-Retinal. J Neurosci 2(15), 4008-4015.
- NEUSC 6040: Cellular and Molecular Neuroscience