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Wolfgang Baehr

Professor of Ophthalmology & Visual Sciences and
Adjunct Professor of Neurobiology & Anatomy and of BiologyBaehr

Diploma University of Heidelberg, Germany

Ph.D. University of Heidelberg, Germany

Research

References

wbaehr@hsc.utah.edu

Wolfgang Baehr's Lab Page

Wolfgang Baehr's PubMed Literature Search

Wolfgang Baehr's Google Scholar Listing

Molecular Biology Program

Vertebrate Phototransduction

Research

Rod and cone photoreceptor cells in the retina mediate vision. These cells have evolved into highly polarized structures consisting of three distinct areas: the outer segment containing membrane disks housing proteins involved in phototransduction, the inner segment where biosynthesis occurs, and the synaptic terminal that transmits excitation by light to downstream neurons. The inner segment (cell body) connects to an outer segment through a narrow 9+0 transition zone and to the synaptic terminal by a slender axon.

Baehr Figure

My laboratory explores mechanisms in membrane protein transport in photoreceptors through the trabsition zone, a homolog of the transition zone in primary cilia. A central question concerns biogenesis and maintenance of the basal body/axoneme cytoskeleton providing the backbone for the outer segment. Defects in basal body docking to the cortex of the inner segment, extension of a transition zone and formation of the axoneme cause a number of non-syndromic and syndromic retinal disorders. Non-syndromic diseases (retinitis pigmentosa, Leber congenital amaurosis) affect only the retina. Syndromes (e.g., Bardet-Biedl, Senior Loken, and Joubert syndrome) affect multiple organs. We are generating knockout and other transgenic mouse models to mimic human retina disease phenotypes and understand mechanisms leading to disease. Finally, we are exploring gene-based and drug-based therapies to rescue the degeneration in our mouse models.

 

Selected Publications, since 2011

  1. Zhang H, Constantine R, et al. (2011) UNC119 regulates G protein trafficking in sensory neurons. Nat Neurosci14, 874-880..
  2. Zhang T, Zhang N, et al. Y (2011). Cone opsin determines the time course of cone photoreceptor degeneration in Leber congenital amaurosis. Proc Natl Acad Sci USA 108, 8879-84.
  3. Zhang Z, He F, et al. (2015). Domain organization and conformational plasticity of the G protein effector, PDE6. J Biol Chem, 290(20):12833-43;
  4. Jiang L, Zhang H et al. (2011). Long-term RNA interference gene therapy in a dominant retinitis pigmentosa mouse model. Proc Natl Acad Sci USA 108, 18476-81.
  5. Constantine R, Zhang H et al. (2012). Uncoordinated(UNC)119: Coordinating the trafficking of myristoylated proteins. Vision Res 75, 26-32.
  6. Ying G, Avasthi P et al. (2014). Centrin 2 is required for mouse olfactory ciliary trafficking and development of ependymal cilia planar polarity. J Neurosci 34, 6377–6388.
  7. Zhang H, Li Jiang L et al. (2014). Mistrafficking of Prenylated Proteins Causes Retinitis Pigmentosa 2. FASEB J, 29(3):932-42..
  8. Jiang L, Wei Y et al. (2015). Heterotrimeric Kinesin-2 (KIF3) mediates transition zone and axoneme formation of mouse photoreceptors. J Biol Chem, 15;290(20):12765-78.
  9. Hanke-Gogokhia C, Wu Z, et al. (2016). Arf-like Protein 3 (ARL3) Regulates Protein Trafficking and Ciliogenesis in Mouse Photoreceptors. J Biol Chem, 291, 7142-7155.
  10. Ying G, Gerstner CD, et al. (2016). Small GTPases Rab8, Rab10 and Rab11 are Dispensable for Rhodopsin Trafficking in Mouse Photoreceptors. Published 16 Aug 2016 PLOS ONEhttp://dx.doi.org/10.1371/journal.pone.0161236.
  11. Ronquillo CC, Hanke-Gogokhia C, et al. (2016). Ciliopathy-associated IQCB1/NPHP5 protein is required for mouse photoreceptor outer segment formation. FASEB J. 30(10):3400-3412.
  12. Hanke-Gogokhia C, Yazigi H, et al. (2017) Arf-like Protein 13b (ARL13b), the ARL3 GEF, regulates assembly of the mouse photoreceptor transition zone. Submitted.

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Last Updated: 7/20/18