Wolfgang Baehr

Summary

Affiliation: University of Utah
Country: USA

Publications

  1. pmc The function of guanylate cyclase 1 and guanylate cyclase 2 in rod and cone photoreceptors
    Wolfgang Baehr
    Department of Ophthalmology and Visual Sciences, University of Utah, Salt Lake City, Utah 84112, USA
    J Biol Chem 282:8837-47. 2007
  2. pmc Naturally occurring animal models with outer retina phenotypes
    Wolfgang 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
  3. pmc Trafficking of membrane-associated proteins to cone photoreceptor outer segments requires the chromophore 11-cis-retinal
    Houbin Zhang
    Department of Ophthalmology, University of Utah Health Science Center, Salt Lake City, Utah 84132, USA
    J Neurosci 28:4008-14. 2008
  4. pmc Trafficking of membrane proteins to cone but not rod outer segments is dependent on heterotrimeric kinesin-II
    Prachee Avasthi
    Department of Ophthalmology, University of Utah Health Science Center, Salt Lake City, Utah 84132, USA
    J Neurosci 29:14287-98. 2009
  5. pmc RNAi-mediated gene suppression in a GCAP1(L151F) cone-rod dystrophy mouse model
    Li Jiang
    Department of Ophthalmology and Visual Sciences, University of Utah Health Science Center, Salt Lake City, Utah, USA
    PLoS ONE 8:e57676. 2013
  6. pmc A model for transport of membrane-associated phototransduction polypeptides in rod and cone photoreceptor inner segments
    Sukanya Karan
    Department of Biology, University of Utah, Salt Lake City, UT 84132, USA
    Vision Res 48:442-52. 2008
  7. pmc cGMP accumulation causes photoreceptor degeneration in CNG channel deficiency: evidence of cGMP cytotoxicity independently of enhanced CNG channel function
    Jianhua Xu
    Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73104, Center for Integrated Protein Science Munich and Department of Pharmacy Center for Drug Research, Ludwig Maximilians Universitat Munchen, 80539 Munich, Germany, John A Moran Eye Center, University of Utah, Salt Lake City, Utah 84132, and Department of Basic Sciences and Pennsylvania College of Optometry, Salus University, Elkins Park, Pennsylvania 19027
    J Neurosci 33:14939-48. 2013
  8. ncbi request reprint Receptor for advanced glycation end products and age-related macular degeneration
    Kimberly 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
  9. ncbi request reprint Identification and light-dependent translocation of a cone-specific antigen, cone arrestin, recognized by monoclonal antibody 7G6
    Houbin 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
  10. pmc A novel GCAP1(N104K) mutation in EF-hand 3 (EF3) linked to autosomal dominant cone dystrophy
    Li Jiang
    Department of Biology, University of Utah, Salt Lake City, UT 84132, USA
    Vision Res 48:2425-32. 2008

Research Grants

Collaborators

Detail Information

Publications57

  1. pmc The function of guanylate cyclase 1 and guanylate cyclase 2 in rod and cone photoreceptors
    Wolfgang 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...
  2. pmc Naturally occurring animal models with outer retina phenotypes
    Wolfgang 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...
  3. pmc Trafficking of membrane-associated proteins to cone photoreceptor outer segments requires the chromophore 11-cis-retinal
    Houbin 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...
  4. pmc Trafficking of membrane proteins to cone but not rod outer segments is dependent on heterotrimeric kinesin-II
    Prachee Avasthi
    Department of Ophthalmology, University of Utah Health Science Center, Salt Lake City, Utah 84132, USA
    J Neurosci 29:14287-98. 2009
    ....
  5. pmc RNAi-mediated gene suppression in a GCAP1(L151F) cone-rod dystrophy mouse model
    Li 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...
  6. pmc A model for transport of membrane-associated phototransduction polypeptides in rod and cone photoreceptor inner segments
    Sukanya 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...
  7. pmc cGMP accumulation causes photoreceptor degeneration in CNG channel deficiency: evidence of cGMP cytotoxicity independently of enhanced CNG channel function
    Jianhua Xu
    Department of Cell Biology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73104, Center for Integrated Protein Science Munich and Department of Pharmacy Center for Drug Research, Ludwig Maximilians Universitat Munchen, 80539 Munich, Germany, John A Moran Eye Center, University of Utah, Salt Lake City, Utah 84132, and Department of Basic Sciences and Pennsylvania College of Optometry, Salus University, Elkins Park, Pennsylvania 19027
    J Neurosci 33:14939-48. 2013
    ..Our results indicate that cGMP accumulation in photoreceptors can itself exert cytotoxic effect in cones, independently of CNG channel activity and Ca(2+) influx. ..
  8. ncbi request reprint Receptor for advanced glycation end products and age-related macular degeneration
    Kimberly 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...
  9. ncbi request reprint Identification and light-dependent translocation of a cone-specific antigen, cone arrestin, recognized by monoclonal antibody 7G6
    Houbin 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...
  10. pmc A novel GCAP1(N104K) mutation in EF-hand 3 (EF3) linked to autosomal dominant cone dystrophy
    Li 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...
  11. ncbi request reprint Retinal remodeling triggered by photoreceptor degenerations
    Bryan 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...
  12. pmc Centrin 2 is required for mouse olfactory ciliary trafficking and development of ependymal cilia planar polarity
    Guoxin Ying
    Departments of Ophthalmology, and Neurobiology and Anatomy, University of Utah Health Science Center, Salt Lake City, Utah 84132, and Departments of Physiology and Biology, University of Utah, Salt Lake City, Utah 84112
    J Neurosci 34:6377-88. 2014
    ....
  13. pmc Uncoordinated (UNC)119: coordinating the trafficking of myristoylated proteins
    Ryan 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...
  14. pmc Proliferative reactive gliosis is compatible with glial metabolic support and neuronal function
    Felix 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
    ....
  15. pmc Cone opsin determines the time course of cone photoreceptor degeneration in Leber congenital amaurosis
    Tao 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...
  16. pmc UNC119 is required for G protein trafficking in sensory neurons
    Houbin 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...
  17. pmc Chemical chaperone TUDCA preserves cone photoreceptors in a mouse model of Leber congenital amaurosis
    Tao 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...
  18. ncbi request reprint Photoreceptor cGMP phosphodiesterase delta subunit (PDEdelta) functions as a prenyl-binding protein
    Houbin 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...
  19. pmc The prenyl-binding protein PrBP/δ: a chaperone participating in intracellular trafficking
    Houbin 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...
  20. pmc Long-term RNA interference gene therapy in a dominant retinitis pigmentosa mouse model
    Li 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...
  21. pmc Novel functions of photoreceptor guanylate cyclases revealed by targeted deletion
    Sukanya 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...
  22. pmc Mutant prominin 1 found in patients with macular degeneration disrupts photoreceptor disk morphogenesis in mice
    Zhenglin 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...
  23. pmc Germline deletion of Cetn1 causes infertility in male mice
    Prachee Avasthi
    Department of Ophthalmology, University of Utah Health Science Center, 65 Mario Capecchi Drive, Salt Lake City, UT 84132, USA
    J Cell Sci 126:3204-13. 2013
    ..These results confirm an essential role for CETN1 in late steps of spermiogenesis and spermatid maturation...
  24. ncbi request reprint 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)...
  25. doi request reprint Involvement of guanylate cyclases in transport of photoreceptor peripheral membrane proteins
    Sukanya Karan
    John A Moran Eye Center, University of Utah, Salt Lake City, UT 84132, USA
    Adv Exp Med Biol 613:351-9. 2008
  26. ncbi request reprint Functional study of photoreceptor PDEdelta
    Houbin Zhang
    Moran Eye Center, University of Utah, Salt Lake City, Utah 84112, USA
    Adv Exp Med Biol 572:485-90. 2006
  27. pmc GCAP1 rescues rod photoreceptor response in GCAP1/GCAP2 knockout mice
    Kim 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...
  28. pmc Retinoid receptors trigger neuritogenesis in retinal degenerations
    Yanhua 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...
  29. pmc GCAP1 mutations associated with autosomal dominant cone dystrophy
    Li 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...
  30. pmc Unc119 gene deletion partially rescues the GRK1 transport defect of Pde6d (- /-) cones
    Houbin Zhang
    Department of Ophthalmology, John A Moran Eye Center, University of Utah Health Science Center, 65 Mario Capecchi Drive, 84132, Salt Lake City, UT, USA
    Adv Exp Med Biol 801:487-93. 2014
    ..These results suggest that cone transport of isoprenylated and acylated proteins is interdependent. ..
  31. ncbi request reprint Light-dependent redistribution of visual arrestins and transducin subunits in mice with defective phototransduction
    Houbin 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...
  32. ncbi request reprint Progressive pathways in age-related macular degeneration
    Kimberly 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
  33. pmc Targeting of mouse guanylate cyclase 1 (Gucy2e) to Xenopus laevis rod outer segments
    Sukanya 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...
  34. pmc Photoreceptor avascular privilege is shielded by soluble VEGF receptor-1
    Ling Luo
    Moran Eye Center, University of Utah, Salt Lake City, United States Department of Ophthalmology, The 306th Hospital of PLA, Beijing, China
    elife 2:e00324. 2013
    ..DOI:http://dx.doi.org/10.7554/eLife.00324.001. ..
  35. pmc Soluble fusion proteins between single transmembrane photoreceptor guanylyl cyclases and their activators
    Izabela 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...
  36. pmc A novel mutation (I143NT) in guanylate cyclase-activating protein 1 (GCAP1) associated with autosomal dominant cone degeneration
    Koji 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...
  37. pmc Rpe65-/- and Lrat-/- mice: comparable models of leber congenital amaurosis
    Jie Fan
    Department of Ophthalmology, Medical University of South Carolina, Charleston, South Carolina 29402, USA
    Invest Ophthalmol Vis Sci 49:2384-9. 2008
    ....
  38. pmc Redundant and unique roles of retinol dehydrogenases in the mouse retina
    Akiko 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...
  39. pmc Role of photoreceptor-specific retinol dehydrogenase in the retinoid cycle in vivo
    Akiko 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...
  40. ncbi request reprint Retinol dehydrogenase (RDH12) protects photoreceptors from light-induced degeneration in mice
    Akiko 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)...
  41. pmc Lecithin-retinol acyltransferase is essential for accumulation of all-trans-retinyl esters in the eye and in the liver
    Matthew 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...
  42. pmc 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)...
  43. pmc Noninvasive two-photon imaging reveals retinyl ester storage structures in the eye
    Yoshikazu 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...
  44. ncbi request reprint The retinoid cycle and retina disease
    Wolfgang Baehr
    Vision Res 43:2957-8. 2003
  45. pmc Annotation and analysis of 10,000 expressed sequence tags from developing mouse eye and adult retina
    Jindan 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)...
  46. pmc Guanylate cyclase-activating protein (GCAP) 1 rescues cone recovery kinetics in GCAP1/GCAP2 knockout mice
    Mark 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...
  47. pmc Characterization of retinal guanylate cyclase-activating protein 3 (GCAP3) from zebrafish to man
    Yoshikazu Imanishi
    Department of Ophthalmology, Pharmacology, and Chemistry, University of Washington, Seattle, WA 98195, USA
    Eur J Neurosci 15:63-78. 2002
    ....
  48. ncbi request reprint Elovl4 mRNA distribution in the developing mouse retina and phylogenetic conservation of Elovl4 genes
    Xian 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...
  49. pmc 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...
  50. pmc Evaluation of the 17-kDa prenyl-binding protein as a regulatory protein for phototransduction in retinal photoreceptors
    Angela 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
    ....
  51. ncbi request reprint Rhodopsin--advances and perspectives
    Kris Palczewski
    Department of Pharmacology, Case Western Reserve University, Cleveland, OH 44106 4965, USA
    Vision Res 46:4425-6. 2006
  52. ncbi request reprint Mutant carbonic anhydrase 4 impairs pH regulation and causes retinal photoreceptor degeneration
    Zhenglin 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...
  53. pmc 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...
  54. pmc Pharmacological and rAAV gene therapy rescue of visual functions in a blind mouse model of Leber congenital amaurosis
    Matthew L Batten
    Department of Ophthalmology, University of Washington, Seattle, Washington, United States of America
    PLoS Med 2:e333. 2005
    ..One cause of LCA is loss of the enzyme lecithin:retinol acyl transferase (LRAT), which is required for regeneration of the visual photopigment in the retina...
  55. ncbi request reprint Assay and functional properties of PrBP(PDEdelta), a prenyl-binding protein interacting with multiple partners
    Houbin 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...
  56. ncbi request reprint Cloning and molecular characterization of cGMP-gated ion channels from rod and cone photoreceptors of striped bass ( M. saxatilis ) retina
    Christophe Paillart
    Department of Physiology, School of Medicine, University of California at San Francisco, San Francisco, California 94143, USA
    Vis Neurosci 23:99-113. 2006
    ....
  57. ncbi request reprint Mouse eye gene microarrays for investigating ocular development and disease
    Rafal 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 Grants21

  1. Mammalian Phototransduction: Role of cGMP and Calcium
    Wolfgang 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. ..
  2. Mammalian Phototransduction: Role of cGMP and Calcium
    Wolfgang 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. ..
  3. Mammalian Phototransduction: Role of cGMP and Calcium
    Wolfgang Baehr; Fiscal Year: 2009
    ..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. ..
  4. Membrane Protein Trafficking in Photoreceptors
    Wolfgang Baehr; Fiscal Year: 2009
    ..Transport of membrane proteins, particularly post-Golgi transport and intraflagellar transport through the cilium, are complex and insufficiently understood in disease etiology. ..
  5. Mammalian Phototransduction: Role of cGMP and Calcium
    Wolfgang Baehr; Fiscal Year: 2010
    ..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. ..
  6. Membrane Protein Trafficking in Photoreceptors
    Wolfgang 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. ..
  7. Mammalian Phototransduction: Role of cGMP and Calcium
    Wolfgang 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. ..
  8. Conference--The Biology and Chemistry of Vision
    Wolfgang 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. ..
  9. PHOTOTRANSDUCTION--METABOLISM OF CGMP AND CALCIUM
    Wolfgang Baehr; Fiscal Year: 1999
    ..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. ..
  10. PHOTOTRANSDUCTION--METABOLISM OF CGMP AND CALCIUM
    Wolfgang Baehr; Fiscal Year: 2000
    ..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. ..
  11. PHOTOTRANSDUCTION--METABOLISM OF CGMP AND CALCIUM
    Wolfgang Baehr; Fiscal Year: 2001
    ..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. ..
  12. PHOTOTRANSDUCTION--METABOLISM OF CGMP AND CALCIUM
    Wolfgang 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. ..
  13. Mammalian Phototransduction: Role of cGMP and Calcium
    Wolfgang 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. ..
  14. Membrane Protein Trafficking in Photoreceptors
    Wolfgang 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. ..