Research Topics
Genomes and Genes
| Wolfgang BaehrSummaryAffiliation: University of Utah Country: USA Publications
Research Grants
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Detail Information
Publications
The function of guanylate cyclase 1 and guanylate cyclase 2 in rod and cone photoreceptorsWolfgang Baehr
Department of Ophthalmology and Visual Sciences, University of Utah, Salt Lake City, Utah 84112, USA
J Biol Chem 282:8837-47. 2007..We interpret these results to demonstrate an intrinsic requirement of GCs for stability and/or transport of a set of membrane-associated phototransduction proteins...- Naturally occurring animal models with outer retina phenotypesWolfgang Baehr
John A Moran Eye Center, Department of Ophthalmology and Visual Sciences, 65 Mario Capecchi Dr, Salt Lake City, UT 84132, USA
Vision Res 49:2636-52. 2009..This article will review the history, phenotypes and gene defects of select animal models with outer retina (photoreceptor and retinal pigment epithelium) degeneration phenotypes... - Trafficking of membrane-associated proteins to cone photoreceptor outer segments requires the chromophore 11-cis-retinalHoubin Zhang
Department of Ophthalmology, University of Utah Health Science Center, Salt Lake City, Utah 84132, USA
J Neurosci 28:4008-14. 2008..Furthermore, the presence of 11-cis-retinal is essential for proper transport of several membrane-associated cone phototransduction polypeptides in these cones... - Trafficking of membrane proteins to cone but not rod outer segments is dependent on heterotrimeric kinesin-IIPrachee Avasthi
Department of Ophthalmology, University of Utah Health Science Center, Salt Lake City, Utah 84132, USA
J Neurosci 29:14287-98. 2009.... - A model for transport of membrane-associated phototransduction polypeptides in rod and cone photoreceptor inner segmentsSukanya Karan
Department of Biology, University of Utah, Salt Lake City, UT 84132, USA
Vision Res 48:442-52. 2008..We conclude that distinct pathways have evolved in rods and cones for transport of integral and peripherally membrane-associated proteins... - A novel GCAP1(N104K) mutation in EF-hand 3 (EF3) linked to autosomal dominant cone dystrophyLi Jiang
Department of Biology, University of Utah, Salt Lake City, UT 84132, USA
Vision Res 48:2425-32. 2008..The rod recovery delays observed in double-flash ERG of affected patients suggest a novel dominant-negative effect that slows GC stimulation... 
Receptor for advanced glycation end products and age-related macular degenerationKimberly A Howes
Moran Eye Center, University of Utah Health Science Center, Salt Lake City, UT 84112, USA
Invest Ophthalmol Vis Sci 45:3713-20. 2004..In the present study receptor for AGE (RAGE)-mediated cellular activation was evaluated in the etiology of human retinal aging and disease...- Retinal remodeling triggered by photoreceptor degenerationsBryan W Jones
John A Moran Eye Center, University of Utah School of Medicine, Salt Lake City, Utah 84132
J Comp Neurol 464:1-16. 2003..Although remodeling may constrain therapeutic intervals for molecular, cellular, or bionic rescue, it suggests that the neural retina may be more plastic than previously believed... - Identification and light-dependent translocation of a cone-specific antigen, cone arrestin, recognized by monoclonal antibody 7G6Houbin Zhang
Department of Ophthalmology, Moran Eye Center, University of Utah Health Sciences Center, Salt Lake City, Utah 84112, USA
Invest Ophthalmol Vis Sci 44:2858-67. 2003..To elucidate the antigen recognized by monoclonal antibody (mAb) 7G6, a widely used cone-specific marker... - Proliferative reactive gliosis is compatible with glial metabolic support and neuronal functionFelix R Vazquez-Chona
Department of Ophthalmology and Visual Sciences, John A Moran Eye Center, University of Utah, 65 Mario Capecchi Dr, Salt Lake City, UT 84132, USA
BMC Neurosci 12:98. 2011.... - Cone opsin determines the time course of cone photoreceptor degeneration in Leber congenital amaurosisTao Zhang
Departments of Ophthalmology and Visual Sciences, University of Utah, Salt Lake City, UT 84132, USA
Proc Natl Acad Sci U S A 108:8879-84. 2011..Our results demonstrate that cone opsins play a major role in determining the degeneration rate of photoreceptors in LCA... - Photoreceptor cGMP phosphodiesterase delta subunit (PDEdelta) functions as a prenyl-binding proteinHoubin Zhang
Department of Neurobiology and Anatomy, University of Utah, Salt Lake City, Utah 84112, USA
J Biol Chem 279:407-13. 2004..06 and 0.70 microm, respectively. Our experiments establish that PDEdelta functions as a prenyl-binding protein interacting with multiple prenylated proteins... - UNC119 is required for G protein trafficking in sensory neuronsHoubin Zhang
Department of Ophthalmology, University of Utah Health Science Center, Salt Lake City, Utah, USA
Nat Neurosci 14:874-80. 2011..UNC119 deletion in both mouse and C. elegans led to G protein mislocalization. Thus, UNC119 is a Gα subunit cofactor essential for G protein trafficking in sensory cilia... - Long-term RNA interference gene therapy in a dominant retinitis pigmentosa mouse modelLi Jiang
Department of Ophthalmology, University of Utah Health Science Center, Salt Lake City, UT 84132, USA
Proc Natl Acad Sci U S A 108:18476-81. 2011..Our results provide a promising strategy toward effective RNAi-based gene therapy by scAAV2/8 delivery for dominant retinal diseases... - Mutant prominin 1 found in patients with macular degeneration disrupts photoreceptor disk morphogenesis in miceZhenglin Yang
Department of Ophthalmology and Visual Science, Eccles Institute of Human Genetics, University of Utah, Salt Lake City, Utah, USA
J Clin Invest 118:2908-16. 2008..Collectively, our results identify what we believe to be a novel complex involved in photoreceptor disk morphogenesis and indicate a possible role for PROM1 and PCDH21 in macular degeneration... - Novel functions of photoreceptor guanylate cyclases revealed by targeted deletionSukanya Karan
Department of Ophthalmology, John A Moran Eye Center, University of Utah Health Science Center, 65 Mario Capecchi Dr, Salt Lake City, UT 84132, USA
Mol Cell Biochem 334:141-55. 2010..In contrast, GC-E(-/-) cones lack proteins of the entire phototransduction cascade. These results suggest that GC-E may participate in transport of peripheral membrane proteins from the endoplasmic reticulum (ER) to the outer segments... - RNAi-mediated gene suppression in a GCAP1(L151F) cone-rod dystrophy mouse modelLi Jiang
Department of Ophthalmology and Visual Sciences, University of Utah Health Science Center, Salt Lake City, Utah, USA
PLoS ONE 8:e57676. 2013..We propose nonallele-specific RNAi knockdown of GCAP1 as a general therapeutic strategy to rescue any GCAP1-based dominant cone-rod dystrophy in human patients... - Uncoordinated (UNC)119: coordinating the trafficking of myristoylated proteinsRyan Constantine
Graduate Program in Neuroscience, University of Utah Health Science Center, Salt Lake City, UT 84132, USA
Vision Res 75:26-32. 2012..These interactions establish UNC119 polypeptides as novel lipid-binding chaperones with specificity for a diverse subset of myristoylated proteins... - Chemical chaperone TUDCA preserves cone photoreceptors in a mouse model of Leber congenital amaurosisTao Zhang
Department of Ophthalmology and Visual Sciences, University of Utah Health Sciences Center, Salt Lake City, Utah 84132, USA
Invest Ophthalmol Vis Sci 53:3349-56. 2012..The purpose of this study is to examine the efficacy of an ER chemical chaperone, tauroursodeoxycholic acid (TUDCA), in preserving cones in the Lrat(-/-) model... - Autosomal dominant cone dystrophy caused by a novel mutation in the GCAP1 gene (GUCA1A)Li Jiang
Department of Ophthalmology and Visual Sciences, University of Utah Health Sciences Center, Salt Lake City, UT 84132, USA
Mol Vis 11:143-51. 2005..To describe the clinical features and genetic analysis of a family with an autosomal dominant cone dystrophy (adCD)... - Functional study of photoreceptor PDEdeltaHoubin Zhang
Moran Eye Center, University of Utah, Salt Lake City, Utah 84112, USA
Adv Exp Med Biol 572:485-90. 2006 - Involvement of guanylate cyclases in transport of photoreceptor peripheral membrane proteinsSukanya Karan
John A. Moran Eye Center, University of Utah, Salt Lake City, UT 84132, USA
Adv Exp Med Biol 613:351-9. 2008 - The prenyl-binding protein PrBP/δ: a chaperone participating in intracellular traffickingHoubin Zhang
Department of Ophthalmology, John A Moran Eye Center, University of Utah Health Science Center, 65 Mario Capecchi Dr, Salt Lake City, UT 84132, USA
Vision Res 75:19-25. 2012..Rod and cone transducin trafficking was largely unaffected. These trafficking defects produce progressive cone-rod dystrophy in the Pde6d(-/-) mouse... - Retinoid receptors trigger neuritogenesis in retinal degenerationsYanhua Lin
Department of Ophthalmology, John A Moran Eye Center, University of Utah, Salt Lake City, UT, USA
FASEB J 26:81-92. 2012..Thus, RXRs are in the final common path and may be therapeutic targets to attenuate retinal remodeling and facilitate global intervention methods in blinding diseases and other neurodegenerative disorders... - GCAP1 mutations associated with autosomal dominant cone dystrophyLi Jiang
Department of Biology, University of Utah, Salt Lake City, UT 84132, USA
Adv Exp Med Biol 664:273-82. 2010..In this article we review these mutations and describe the consequences of specific mutations on GCAP1 structure and GC stimulation... - Targeting of mouse guanylate cyclase 1 (Gucy2e) to Xenopus laevis rod outer segmentsSukanya Karan
Department of Ophthalmology, John A Moran Eye Center, University of Utah Health Science Center, Salt Lake City, UT 84132, USA
Vision Res 51:2304-11. 2011..Our results suggest targeting is due to either multiple weak signals in the cytoplasmic domain of GC1, or co-transport to the OS with an accessory protein... - Progressive pathways in age-related macular degenerationKimberly A Howes
Moran Eye Center, University of Utah Health Science Center, Salt Lake City, UT 84112, USA
Adv Exp Med Biol 533:91-6. 2003 - Light-dependent redistribution of visual arrestins and transducin subunits in mice with defective phototransductionHoubin Zhang
Department of Neurobiology and Anatomy, University of Utah, Salt Lake City, USA
Mol Vis 9:231-7. 2003..The underlying translocation mechanisms are unclear. This study examines these previously demonstrated translocation in mice with defective phototransduction... - GCAP1 rescues rod photoreceptor response in GCAP1/GCAP2 knockout miceKim A Howes
Department of Ophthalmology, Moran Eye Center, University of Utah Health Science Center, Salt Lake City, UT 84112 5330, USA
EMBO J 21:1545-54. 2002..These results demonstrate that GCAP1 at near normal levels supports the generation of wild-type flash responses in the absence of GCAP2... - Rpe65-/- and Lrat-/- mice: comparable models of leber congenital amaurosisJie Fan
Department of Ophthalmology, Medical University of South Carolina, Charleston, South Carolina 29402, USA
Invest Ophthalmol Vis Sci 49:2384-9. 2008.... - Soluble fusion proteins between single transmembrane photoreceptor guanylyl cyclases and their activatorsIzabela Sokal
Department of Ophthalmology, University of Washington, Seattle, Washington 98195-6485, USA
Biochemistry 41:251-7. 2002..Thus, fusion proteins may provide an important advance for further structural studies of photoreceptor GCs and other sTMRs with and without different forms of regulatory proteins... - A novel mutation (I143NT) in guanylate cyclase-activating protein 1 (GCAP1) associated with autosomal dominant cone degenerationKoji M Nishiguchi
Ocular Molecular Genetics Institute, Massachusetts Eye and Ear Infirmary, Boston, MA, USA
Invest Ophthalmol Vis Sci 45:3863-70. 2004..To identify pathogenic mutations in the guanylate cyclase-activating protein 1 (GCAP1) and GCAP2 genes and to characterize the biochemical effect of mutation on guanylate cyclase (GC) stimulation... - Lecithin-retinol acyltransferase is essential for accumulation of all-trans-retinyl esters in the eye and in the liverMatthew L Batten
Department of Ophthalmology, University of Washington, Seattle, Washington 98195, USA
J Biol Chem 279:10422-32. 2004..We conclude that Lrat-/- mice may serve as an animal model with early onset severe retinal dystrophy and severe retinyl ester deprivation... - Retinol dehydrogenase (RDH12) protects photoreceptors from light-induced degeneration in miceAkiko Maeda
Department of Pharmacology and Ophthalmology, Case Western Reserve University, Cleveland, Ohio 44106, USA
J Biol Chem 281:37697-704. 2006..Thus, severe visual impairments of individuals with null mutations in RDH12 may likely be caused by light damage(1)... - Redundant and unique roles of retinol dehydrogenases in the mouse retinaAkiko Maeda
Department of Pharmacology, Case Western Reserve University, Cleveland, OH 44106 4965, USA
Proc Natl Acad Sci U S A 104:19565-70. 2007..The results suggest that RDH8 and RDH12 are dispensable in support of the visual cycle but appear to be key components in clearance of free all-trans-retinal, thereby preventing A2E accumulation and photoreceptor cell death... - Role of photoreceptor-specific retinol dehydrogenase in the retinoid cycle in vivoAkiko Maeda
Department of Ophthalmology, University of Washington, Seattle, Washington 98195 6485, USA
J Biol Chem 280:18822-32. 2005..We conclude that prRDH is an enzyme that catalyzes reduction of all-trans-retinal in the rod outer segment, most noticeably at higher light intensities and prolonged illumination, but is not an essential enzyme of the retinoid cycle... - A novel GCAP1 missense mutation (L151F) in a large family with autosomal dominant cone-rod dystrophy (adCORD)Izabela Sokal
Department of Ophthalmology, University of Washington, Seattle, Washington, USA
Invest Ophthalmol Vis Sci 46:1124-32. 2005..To elucidate the phenotypic and biochemical characteristics of a novel mutation associated with autosomal dominant cone-rod dystrophy (adCORD)... - The retinoid cycle and retina diseaseWolfgang Baehr
Vision Res 43:2957-8. 2003 - Annotation and analysis of 10,000 expressed sequence tags from developing mouse eye and adult retinaJindan Yu
Ophthalmology and Visual Science, University of Michigan, 1000 Wall Street, Ann Arbor, MI 48105, USA
Genome Biol 4:R65. 2003..We have generated and analyzed 10,000 expressed sequence tags (ESTs) from three mouse eye tissue cDNA libraries: embryonic day 15.5 (M15E) eye, postnatal day 2 (M2PN) eye and adult retina (MRA)... - Elovl4 mRNA distribution in the developing mouse retina and phylogenetic conservation of Elovl4 genesXian Mei Zhang
Department of Ophthalmology and Jules Stein Eye Institute, UCLA School of Medicine, USA
Mol Vis 9:301-7. 2003..The purpose of this study is to characterize cross-species conservation of ELOVL4 and investigate its mRNA distribution in the developing mouse eye... - Guanylate cyclase-activating protein (GCAP) 1 rescues cone recovery kinetics in GCAP1/GCAP2 knockout miceMark E Pennesi
Department of Ophthalmology, Cullen Eye Institute, Baylor College of Medicine, Houston, TX 77030, USA
Proc Natl Acad Sci U S A 100:6783-8. 2003..These studies reveal that, similar to rods, deletion of GCAP1 and GCAP2 delays the recovery of light responses in cones and GCAP1 restores the recovery of cone responses in the absence of GCAP2... - Characterization of retinal guanylate cyclase-activating protein 3 (GCAP3) from zebrafish to manYoshikazu Imanishi
Department of Ophthalmology, Pharmacology, and Chemistry, University of Washington, Seattle, WA 98195, USA
Eur J Neurosci 15:63-78. 2002.... - Noninvasive two-photon imaging reveals retinyl ester storage structures in the eyeYoshikazu Imanishi
Department of Ophthalmology, University of Washington, 1959 NE Pacific St, Box 356485, Seattle, WA 98195-6485, USA
J Cell Biol 164:373-83. 2004..These results indicate that RESTs located close to the RPE plasma membrane are essential components in 11-cis-retinal production... - Diversity of guanylate cyclase-activating proteins (GCAPs) in teleost fish: characterization of three novel GCAPs (GCAP4, GCAP5, GCAP7) from zebrafish (Danio rerio) and prediction of eight GCAPs (GCAP1-8) in pufferfish (Fugu rubripes)Yoshikazu Imanishi
Department of Ophthalmology, University of Washington, Seattle, WA 98195, USA
J Mol Evol 59:204-17. 2004..Based on genome searches and EST analyses, the mouse and human genomes do not harbor GCAP4-8 or GCIP genes... - Evaluation of the 17-kDa prenyl-binding protein as a regulatory protein for phototransduction in retinal photoreceptorsAngela W Norton
Department of Biochemistry and Molecular Biology, University of New Hampshire, Durham, New Hampshire 03824-2617, USA
J Biol Chem 280:1248-56. 2005.... - Rhodopsin--advances and perspectivesKris Palczewski
Department of Pharmacology, Case Western Reserve University, Cleveland, OH 44106-4965, USA
Vision Res 46:4425-6. 2006 - Mutant carbonic anhydrase 4 impairs pH regulation and causes retinal photoreceptor degenerationZhenglin Yang
Department of Ophthalmology and Visual Science, University of Utah Health Science Center, Salt Lake City 84112, USA
Hum Mol Genet 14:255-65. 2005..The importance of a functional CA4 for survival of photoreceptors implies that CA inhibitors, which are widely used as medications, particularly in the treatment of glaucoma, may have long-term adverse effects on vision... - Retinoid absorption and storage is impaired in mice lacking lecithin:retinol acyltransferase (LRAT)Sheila M O'Byrne
Institute of Human Nutrition, College of Physicians and Surgeons, Columbia University, New York, New York 10032, USA
J Biol Chem 280:35647-57. 2005..The fatty acyl composition of these (chylomicron) retinyl esters suggests that they are synthesized via an acyl-CoA-dependent process suggesting the existence of a physiologically significant acyl-CoA:retinol acyltransferase... - Pharmacological and rAAV gene therapy rescue of visual functions in a blind mouse model of Leber congenital amaurosisMatthew L Batten
Department of Ophthalmology, University of Washington, Seattle, Washington, United States of America
PLoS Med 2:e333. 2005..These complementary methods offer hope of developing treatment to restore vision in humans with certain forms of hereditary congenital blindness... - Assay and functional properties of PrBP(PDEdelta), a prenyl-binding protein interacting with multiple partnersHoubin Zhang
Methods Enzymol 403:42-56. 2005..In mammalian photoreceptors, the results are consistent with a role of PrBP(PDEdelta) in the transport of prenylated proteins from their site of synthesis in the inner segment to the outer segment where phototransduction occurs... - Cloning and molecular characterization of cGMP-gated ion channels from rod and cone photoreceptors of striped bass ( M. saxatilis ) retinaChristophe Paillart
Department of Physiology, School of Medicine, University of California at San Francisco, San Francisco, California 94143, USA
Vis Neurosci 23:99-113. 2006.... - Mouse eye gene microarrays for investigating ocular development and diseaseRafal Farjo
Department of Ophthalmology, W.K. Kellogg Eye Center, University of Michigan, 1000 Wall Street, Ann Arbor, MI 48105, USA
Vision Res 42:463-70. 2002..The I-gene microarrays will be useful for establishing expression profiles of the mouse eye/retina and provide a resource for defining molecular pathways involved in development, aging and disease...
Research Grants
- Mammalian Phototransduction: Role of cGMP and CalciumWolfgang Baehr; Fiscal Year: 2006..Taken together, this research will enhance our knowledge of the structure and function of components involved in the regulation of cGMP and Ca 2+, two important secondary messengers of phototransduction. ..
- Mammalian Phototransduction: Role of cGMP and CalciumWolfgang Baehr; Fiscal Year: 2007..The proposed research will contribute to our understanding of the cGMP/Ca2+ feed back system in photoreceptors, as well mechanisms leading to retina disease based on GCAP mutations. ..
- Membrane Protein Trafficking in PhotoreceptorsWolfgang Baehr; Fiscal Year: 2010..Transport of membrane proteins, particularly post-Golgi transport and intraflagellar transport through the cilium, are complex and insufficiently understood in disease etiology. ..
- Mammalian Phototransduction: Role of cGMP and CalciumWolfgang Baehr; Fiscal Year: 2011..The proposed research will contribute to our understanding of the cGMP/Ca2+ feed back system in photoreceptors, as well mechanisms leading to retina disease based on GCAP mutations. ..
- Conference--The Biology and Chemistry of VisionWolfgang Baehr; Fiscal Year: 2003..Proposed sessions include topics on the biology and physiology of photoreceptors, human genetics and retinal disease, as well as gene therapy of recessive and dominant dystrophies. ..
- PHOTOTRANSDUCTION--METABOLISM OF CGMP AND CALCIUMWolfgang Baehr; Fiscal Year: 2002..Collectively, this research will enhance fundamental knowledge of structure/function relationships of components participating in the regulation of cGMP and Ca2+, two important second messengers of phototransduction. ..
- Mammalian Phototransduction: Role of cGMP and CalciumWolfgang Baehr; Fiscal Year: 2003..Taken together, this research will enhance our knowledge of the structure and function of components involved in the regulation of cGMP and Ca 2+, two important secondary messengers of phototransduction. ..
- Membrane Protein Trafficking in PhotoreceptorsWolfgang Baehr; Fiscal Year: 2011..Transport of membrane proteins, particularly post-Golgi transport and intraflagellar transport through the cilium, are complex and insufficiently understood in disease etiology. ..