The Baehr Laboratory

Baehr

Wolfgang Baehr was born in Mannheim, Germany, and studied organic chemistry at the University of Heidelberg. His career in retinal research was launched in the Department of Biochemistry, Princeton University, in 1976. Dr. Baehr was recruited from the Cullen Eye Institute at Baylor College of Medicine where he was a Jules and Doris Stein Research to Prevent Blindness Professor from 1987—1994, and joined the University of Utah Moran Eye Center as Professor of Ophthalmology and Director of our Foundation Fighting Blindness Center.

Dr. Baehr’s career work addresses the bio-chemistry and molecular biology of the capture of light by photoreceptors in the eye (phototransduction), and the biochemistry of the key elements in that process (the Visual Cycle) with a focus on gene defects causative for human retinal disease. Dr. Baehr’s early research generated one of the first transgenic mouse models for autosomal dominant retinitis pigmentosa, identified specific gene defects in several other animal models of human disease, and characterized key regulatory molecules in the phototransduction pathway. Dr. Baehr has published over 145 manuscripts covering topics in inorganic and organic chemistry, biophysics, biochemistry, molecular biology, bacteriology, infectious disease and genetics.

Chart of the Visual Cycle

The Visual Cycle

At the Moran Eye Center, Dr. Baehr’s laboratory continues the hunt for genes implicated in blinding retinal degenerations using techniques from molecular biology, biochemistry, and neurobiology. In addition to the study of the 50-60 genes thought to be involved in rod and cone phototransduction, the laboratory is also tracking genes involved in the Visual Cycle. This pathway recycles nutritionally-derived Vitamin A aldehyde, the light-catching molecule or chromophore of photoreceptor cells, employing a complex export/import process between photoreceptors and the retinal pigmented epithelium.

Education: Ph.D., University of Heidelberg, Germany

Academic Appointments: Ralph and Mary Tuck Professor of Ophthalmology & Visual Sciences; Director, Foundation Fighting Blindness Center at the John A. Moran Eye Center; Adjunct Professor of Neurobiology and Anatomy; Adjunct Professor of Biology

Patient Care Significance

Photo of cone photoreceptors

Cone photoreceptors

The long-range goal of Dr. Baehr’s research is to design strategies to ameliorate or cure human retinal degenerations: diseases for which no cures exist. These strategies are based on the discovery of many genes and their related proteins that malfunction in inherited retinal diseases. The complexities of these pathways means that designing curative gene therapies is a complex process requiring profound and accurate knowledge of the molecules involved and their many interactions. Dr. Baehr arguably heads the world’s premier molecular genetics laboratory: the Moran Eye Center’s lead team in discovering the mechanisms underlying incurable retinal diseases.

Diagram of the transduction of light to chemical signals in the retinal photoreceptor cells

The transduction of light to chemical signals in the retinal photoreceptor cells.

A sample of major publications from the Baehr Laboratory

Nishiguchi K. N., Sokal I., Yang L., Roychowdhury N., Palczewski K., Berson E. L., Dryja T. P., and Baehr W. (2004) A Novel (I143NT) Mutation in Guanylate Cyclase-Activating Protein 1 (GCAP1) Associated with Autosomal Dominant Cone Dystrophy. Invest. Ophthalmol. & Visual Science, 45:3863-3870.

Imanishi Y., Batten M. L., Piston D. W., Baehr W., and Palczewski K. (2004) Non-invasive two-photon imaging reveals vitamin A storage structures, in the eye. J. Cell Biol., 164:373-83.

Zhang H., Liu X-H., Zhang K., Chen C.K., Frederick J. M., Prestwich, G. D., and Baehr, W. (2004) Photoreceptor cGMP Phosphodiesterase d-Subunit (PDEd) Functions as a Prenyl Binding Protein. J. Biol. Chem. 279:407-13. Epub 2003 Oct 15.

Batten M. L., Imanishi Y., Maeda T., Tu D., Moise A. R., Bronson D., Possin D., Van Gelder R. N., Baehr W., and Palczewski K. (2004) Lecithin: retinol acyltransferase (LRAT) is Essential for Accumulation of Retinyl Esters in the Eye and in the Liver. J. Biol. Chem., 279: 10422-10432. Epub 2003 Dec 18.

Howes K., Pennesi M. E., Sokal I., Church-Kopish J., Schmidt B., Margolis P., Frederick J. M., Rieke F. M., Palczewski K., Wu S. M., Detwiler P., and Baehr W. (2002) GCAP1 rescues rod photoreceptor response in GCAP1/2 knockout mice. The EMBO Journal, 21:1545-1554.