Alan D Marmorstein

Summary

Affiliation: Cleveland Clinic Foundation
Country: USA

Publications

  1. ncbi Lack of fibulin-3 causes early aging and herniation, but not macular degeneration in mice
    Precious J McLaughlin
    Department of Ophthalmology and Vision Science, University of Arizona, Tucson, AZ, USA
    Hum Mol Genet 16:3059-70. 2007
  2. pmc Bestrophin, the product of the Best vitelliform macular dystrophy gene (VMD2), localizes to the basolateral plasma membrane of the retinal pigment epithelium
    A D Marmorstein
    Department of Ophthalmic Research, Cole Eye Institute, and Department of Cell Biology, Lerner Research Institute, The Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland OH 44195, USA
    Proc Natl Acad Sci U S A 97:12758-63. 2000
  3. ncbi The polarity of the retinal pigment epithelium
    A D Marmorstein
    Department of Ophthalmic Research, Cole Eye Institute, Cleveland Clinic Foundation i31, 9500 Euclid Avenue, Cleveland, Ohio, 44195, USA
    Traffic 2:867-72. 2001
  4. ncbi In vivo gene transfer as a means to study the physiology and morphogenesis of the retinal pigment epithelium in the rat
    Alan D Marmorstein
    Department of Ophthalmic Research, Cole Eye Institute, i31, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA
    Methods 30:277-85. 2003
  5. ncbi Spectral profiling of autofluorescence associated with lipofuscin, Bruch's Membrane, and sub-RPE deposits in normal and AMD eyes
    Alan D Marmorstein
    Department of Ophthalmic Research, Cole Eye Institute, The Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA
    Invest Ophthalmol Vis Sci 43:2435-41. 2002
  6. pmc Hydrodynamic properties of porcine bestrophin-1 in Triton X-100
    J Brett Stanton
    Department of Ophthalmology and Vision Science, University of Arizona, Tucson, AZ 85711, and Cole Eye Institute, Cleveland Clinic Foundation, OH 44195, USA
    Biochim Biophys Acta 1758:241-7. 2006
  7. pmc Contribution of Kir4.1 to the mouse electroretinogram
    Jiang Wu
    Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, OH 44195, USA
    Mol Vis 10:650-4. 2004
  8. ncbi Noninvasive recording and response characteristics of the rat dc-electroretinogram
    Neal S Peachey
    Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, OH 44195, USA
    Vis Neurosci 19:693-701. 2002
  9. pmc Light-evoked responses of the mouse retinal pigment epithelium
    Jiang Wu
    Cole Eye Institute, Cleveland Clinic Foundation, Cleveland 44195, USA
    J Neurophysiol 91:1134-42. 2004
  10. pmc Voltage-dependent calcium channel CaV1.3 subunits regulate the light peak of the electroretinogram
    Jiang Wu
    Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, OH 44195, USA
    J Neurophysiol 97:3731-5. 2007

Collaborators

Detail Information

Publications26

  1. ncbi Lack of fibulin-3 causes early aging and herniation, but not macular degeneration in mice
    Precious J McLaughlin
    Department of Ophthalmology and Vision Science, University of Arizona, Tucson, AZ, USA
    Hum Mol Genet 16:3059-70. 2007
    ..These data demonstrate that fibulin-3 plays an important role in maintaining the integrity of fascia connective tissues and regulates aging...
  2. pmc Bestrophin, the product of the Best vitelliform macular dystrophy gene (VMD2), localizes to the basolateral plasma membrane of the retinal pigment epithelium
    A D Marmorstein
    Department of Ophthalmic Research, Cole Eye Institute, and Department of Cell Biology, Lerner Research Institute, The Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland OH 44195, USA
    Proc Natl Acad Sci U S A 97:12758-63. 2000
    ..The basolateral plasma membrane localization of bestrophin suggests the possibility that bestrophin plays a role in generating the altered electrooculogram of individuals with Best disease...
  3. ncbi The polarity of the retinal pigment epithelium
    A D Marmorstein
    Department of Ophthalmic Research, Cole Eye Institute, Cleveland Clinic Foundation i31, 9500 Euclid Avenue, Cleveland, Ohio, 44195, USA
    Traffic 2:867-72. 2001
    ....
  4. ncbi In vivo gene transfer as a means to study the physiology and morphogenesis of the retinal pigment epithelium in the rat
    Alan D Marmorstein
    Department of Ophthalmic Research, Cole Eye Institute, i31, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA
    Methods 30:277-85. 2003
    ..In addition, there are substantial savings with respect to time and money in comparison to standard transgenic approaches...
  5. ncbi Spectral profiling of autofluorescence associated with lipofuscin, Bruch's Membrane, and sub-RPE deposits in normal and AMD eyes
    Alan D Marmorstein
    Department of Ophthalmic Research, Cole Eye Institute, The Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA
    Invest Ophthalmol Vis Sci 43:2435-41. 2002
    ....
  6. pmc Hydrodynamic properties of porcine bestrophin-1 in Triton X-100
    J Brett Stanton
    Department of Ophthalmology and Vision Science, University of Arizona, Tucson, AZ 85711, and Cole Eye Institute, Cleveland Clinic Foundation, OH 44195, USA
    Biochim Biophys Acta 1758:241-7. 2006
    ..This stoichiometry differs from that previously measured for recombinant best-1, suggesting that further studies are necessary to determine the stoichiometry of functional best-1 in RPE membranes...
  7. pmc Contribution of Kir4.1 to the mouse electroretinogram
    Jiang Wu
    Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, OH 44195, USA
    Mol Vis 10:650-4. 2004
    ..The electroretinogram (ERG) represents the combination of several distinct cellular processes and conductances. Here, we define the contribution that K+ conductance through Kir4.1 channels makes to the mouse ERG...
  8. ncbi Noninvasive recording and response characteristics of the rat dc-electroretinogram
    Neal S Peachey
    Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, OH 44195, USA
    Vis Neurosci 19:693-701. 2002
    ....
  9. pmc Light-evoked responses of the mouse retinal pigment epithelium
    Jiang Wu
    Cole Eye Institute, Cleveland Clinic Foundation, Cleveland 44195, USA
    J Neurophysiol 91:1134-42. 2004
    ..Further studies of mutant mice will allow us to define the functional consequences of gene manipulation on RPE function and to evaluate specific hypotheses regarding the generation of ERG components...
  10. pmc Voltage-dependent calcium channel CaV1.3 subunits regulate the light peak of the electroretinogram
    Jiang Wu
    Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, OH 44195, USA
    J Neurophysiol 97:3731-5. 2007
    ..3(-/-) littermates. Our results identify Ca(V)1.3 as the principal pore-forming subunit of VDCCs involved in stimulating the ERG LP...
  11. pmc Tubby-like protein 1 (TULP1) interacts with F-actin in photoreceptor cells
    Quansheng Xi
    Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA
    Invest Ophthalmol Vis Sci 46:4754-61. 2005
    ..Toward a better understanding of the role of TULP1 in retinal disease, its subcellular localization was sought and the TULP1 protein binding partners identified...
  12. ncbi Protein database, human retinal pigment epithelium
    Karen A West
    Cole Eye Institute and Lerner Research Institute, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA
    Mol Cell Proteomics 2:37-49. 2003
    ..Preliminary analyses have identified 278 proteins and provide a starting point for building a database of the human RPE proteome...
  13. pmc Functional abnormalities in the retinal pigment epithelium of CFTR mutant mice
    Jiang Wu
    Cole Eye Institute, Cole Eye Institute I 31, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA
    Exp Eye Res 83:424-8. 2006
    ....
  14. ncbi Retinal pigment epithelial cells exhibit unique expression and localization of plasma membrane syntaxins which may contribute to their trafficking phenotype
    Seng Hui Low
    Department of Cell Biology, Lerner Research Institute, Cleveland, Ohio 44195, USA
    J Cell Sci 115:4545-53. 2002
    ..We propose that regulation of the expression and subcellular localization of plasma membrane SNAREs may be a general mechanism that contributes to the establishment of distinct sorting phenotypes among epithelial cell types...
  15. pmc Aberrant accumulation of EFEMP1 underlies drusen formation in Malattia Leventinese and age-related macular degeneration
    Lihua Y Marmorstein
    Cole Eye Institute, and Department of Cell Biology, Lerner Research Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA
    Proc Natl Acad Sci U S A 99:13067-72. 2002
    ..These data present evidence that misfolding and aberrant accumulation of EFEMP1 may cause drusen formation and cellular degeneration and play an important role in the etiology of both ML and AMD...
  16. ncbi Bestrophin interacts physically and functionally with protein phosphatase 2A
    Lihua Y Marmorstein
    Department of Ophthalmic Research, Cole Eye Institute, The Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA
    J Biol Chem 277:30591-7. 2002
    ..These data suggest that bestrophin is in the signal transduction pathway that modulates the light peak of the electrooculogram, that it is regulated by phosphorylation, and that phosphorylation of bestrophin is in turn regulated by PP2A...
  17. ncbi Proteome survey of proliferating and differentiating rat RPE-J cells
    K A West
    Cole Eye Institute, Lerner Research Institute, Cleveland Clinic Foundation, 9500 Euclid Ave, Cleveland, OH 44195, USA
    Exp Eye Res 73:479-91. 2001
    ....
  18. ncbi Oxidized low density lipoprotein-induced inhibition of processing of photoreceptor outer segments by RPE
    G Hoppe
    Department of Cell Biology, Lerner Research Institute, The Cleveland Clinic Foundation, Ohio 44195, USA
    Invest Ophthalmol Vis Sci 42:2714-20. 2001
    ..CONCLUSIONS: OxLDL induces a reduction in the processing of OS by RPE by perturbing the fusion of lysosomes with phagosomes...
  19. ncbi Formation and progression of sub-retinal pigment epithelium deposits in Efemp1 mutation knock-in mice: a model for the early pathogenic course of macular degeneration
    Lihua Y Marmorstein
    Department of Ophthalmology and Vision Science, University of Arizona, Tucson, AZ 85711, USA
    Hum Mol Genet 16:2423-32. 2007
    ..We conclude that these mice are a valuable tool for studying the primary pathogenic course of basal deposits associated with macular degeneration and for testing prevention and treatment strategies for this class of diseases...
  20. ncbi The challenge of modeling macular degeneration in mice
    Alan D Marmorstein
    Department of Ophthalmology and Vision Science, University of Arizona, 655 North Alvernon Way, Suite 108, Tucson, AZ 85711, USA
    Trends Genet 23:225-31. 2007
    ..By modeling MD in mice, we can learn about aspects of MD that an animal with a macula would be unable to teach us...
  21. pmc Focus on molecules: bestrophin (best-1)
    Alan D Marmorstein
    Department of Ophthalmology and Vision Science, University of Arizona, 655 N Alvernon Way, Suite 108, Tucson, AZ 85711, USA
    Exp Eye Res 85:423-4. 2007
  22. pmc The light peak of the electroretinogram is dependent on voltage-gated calcium channels and antagonized by bestrophin (best-1)
    Lihua Y Marmorstein
    Department of Ophthalmology and Vision Science, University of Arizona, Tucson 85711, USA
    J Gen Physiol 127:577-89. 2006
    ..Furthermore, we suggest that the loss of vision associated with BMD is not caused by the same pathologic process as the diminished LP, but rather is caused by as yet unidentified effects of best-1 on other cellular processes...
  23. pmc Bestrophin-2 is involved in the generation of intraocular pressure
    Benjamin Bakall
    Department of Ophthalmology and Vision Science, University of Arizona, Tucson, Arizona
    Invest Ophthalmol Vis Sci 49:1563-70. 2008
    ..Mutations in bestrophin-1 (Best1) cause several blinding eye diseases, but little is known about other bestrophin family members. This study involved disruption of the Best2 gene in mice...
  24. pmc A model of best vitelliform macular dystrophy in rats
    Alan D Marmorstein
    Department of Ophthalmology, University of Arizona College of Medicine, Tucson, Arizona 85711, USA
    Invest Ophthalmol Vis Sci 45:3733-9. 2004
    ..Bestrophin is thought to be the Cl channel that generates the LP. The goal was to generate an animal model of BMD and to determine the effects of bestrophin overexpression on the RPE-generated components of the ERG...
  25. ncbi Expression and localization of bestrophin during normal mouse development
    Benjamin Bakall
    Department of Genetics and Pathology, Rudbeck Laboratory, Uppsala University, Uppsala, Sweden
    Invest Ophthalmol Vis Sci 44:3622-8. 2003
    ..The purpose of this study was to determine the postnatal onset of expression of bestrophin mRNA and protein in the mouse retinal pigment epithelium (RPE)...
  26. pmc Targeted disruption of fibulin-4 abolishes elastogenesis and causes perinatal lethality in mice
    Precious J McLaughlin
    Department of Ophthalmology and Vision Science, University of Arizona, Tucson, AZ 85711, USA
    Mol Cell Biol 26:1700-9. 2006
    ..In addition, fibulin-4 strongly interacts with tropoelastin and colocalizes with elastic fibers in culture. These results demonstrate that fibulin-4 plays an irreplaceable role in elastogenesis...