ROBERT MOLDAY

Summary

Affiliation: University of British Columbia
Country: Canada

Publications

  1. pmc Subretinal gene delivery using helper-dependent adenoviral vectors
    Linda Wu
    Physiology and Experimental Medicine Program, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G1X8, Canada
    Cell Biosci 1:15. 2011
  2. ncbi request reprint ATP-binding cassette transporter ABCA4: molecular properties and role in vision and macular degeneration
    Robert S Molday
    Department of Biochemistry and Molecular Biology, Centre for Macular Research, University of British Columbia, 2350 Health Sciences Mall, Vancouver, BC, V6T 1Z3, Canada
    J Bioenerg Biomembr 39:507-17. 2007
  3. ncbi request reprint Retinoschisin (RS1), the protein encoded by the X-linked retinoschisis gene, is anchored to the surface of retinal photoreceptor and bipolar cells through its interactions with a Na/K ATPase-SARM1 complex
    Laurie L Molday
    Department of Biochemistry and Molecular Biology, Centre for Macular Research, University of British Columbia, Vancouver, British Columbia, Canada
    J Biol Chem 282:32792-801. 2007
  4. pmc The role of the photoreceptor ABC transporter ABCA4 in lipid transport and Stargardt macular degeneration
    Robert S Molday
    Department of Biochemistry, Centre for Macular Research, University of British Columbia, Vancouver, B C, Canada V6T 1Z3
    Biochim Biophys Acta 1791:573-83. 2009
  5. ncbi request reprint Focus on molecules: retinoschisin (RS1)
    Robert S Molday
    Department of Biochemistry, Centre for Macular Research, University of British Columbia, Vancouver, BC V6T 1Z3 Canada
    Exp Eye Res 84:227-8. 2007
  6. pmc Defective lipid transport and biosynthesis in recessive and dominant Stargardt macular degeneration
    Robert S Molday
    Department of Biochemistry and Molecular Biology, Centre of Macular Research, University of British Columbia, 2350 Health Sciences Mall, Vancouver, BC, Canada
    Prog Lipid Res 49:476-92. 2010
  7. ncbi request reprint RS1, a discoidin domain-containing retinal cell adhesion protein associated with X-linked retinoschisis, exists as a novel disulfide-linked octamer
    Winco W H Wu
    Department of Biochemistry and Molecular Biology, University of British Columbia, Vancouver, British Columbia, V6T 1Z3, Canada
    J Biol Chem 280:10721-30. 2005
  8. ncbi request reprint Disease mechanisms and gene therapy in a mouse model for X-linked retinoschisis
    Laurie L Molday
    University of British Columbia, Vancouver, BC
    Adv Exp Med Biol 572:283-9. 2006
  9. ncbi request reprint Binding of N-retinylidene-PE to ABCA4 and a model for its transport across membranes
    Robert S Molday
    University of British Columbia, Vancouver, BC
    Adv Exp Med Biol 572:465-70. 2006
  10. pmc In vivo imaging of the mouse model of X-linked juvenile retinoschisis with fourier domain optical coherence tomography
    Jing Xu
    School of Engineering Science, Simon Fraser University, Burnaby, British Columbia, Canada
    Invest Ophthalmol Vis Sci 50:2989-93. 2009

Collaborators

  • Kang Zhang
  • Ming Zhong
  • Seok H Min
  • Heidi Stohr
  • Alessandro Iannaccone
  • Daniel M Hornan
  • Monica M Jablonski
  • Laurie L Molday
  • Winco W H Wu
  • Frank M Dyka
  • Bernhard H F Weber
  • Michael C M Kwok
  • Julie P Wong
  • Andreas Janssen
  • Mathias W Seeliger
  • Linda Wu
  • Seelochan Beharry
  • Jing Xu
  • Jonathan A Coleman
  • William W Hauswirth
  • Daniel Mordes
  • Juergen Kast
  • Paulo J C Lin
  • Celene Grayson
  • Jinhi Ahn
  • Haining Zhong
  • Rene Warren
  • Jim Hu
  • Rui Guan
  • Simon Lam
  • Rongqi Duan
  • Derek van der Kooy
  • Huibi Cao
  • Rod Bremner
  • Marinko V Sarunic
  • Emmanuelle Reboul
  • Naoyuki Tanimoto
  • Thomas A Grigliatti
  • Leonard J Foster
  • Tom A Pfeifer
  • Juha M Holopainen
  • Mariko Kawada
  • Jane Y Wu
  • Lili Xu
  • Liya Yuan
  • Astra Dinculescu
  • Kristiane Hudl
  • Felix Tonagel
  • Adrian M Timmers
  • John Orlowski
  • Masayuki Numata
  • Warren P Williams
  • Yvonne Luu
  • Karen L White
  • Heinrich Schrewe
  • Ernst Tamm
  • Gesine B Jaissle
  • Christoph Friedburg
  • Andrea Gehrig
  • King Wai Yau

Detail Information

Publications32

  1. pmc Subretinal gene delivery using helper-dependent adenoviral vectors
    Linda Wu
    Physiology and Experimental Medicine Program, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G1X8, Canada
    Cell Biosci 1:15. 2011
    ..Since these vectors have a large cloning capacity, they have great potential to extend the success of gene therapy achieved using the adeno-associated viral vector...
  2. ncbi request reprint ATP-binding cassette transporter ABCA4: molecular properties and role in vision and macular degeneration
    Robert S Molday
    Department of Biochemistry and Molecular Biology, Centre for Macular Research, University of British Columbia, 2350 Health Sciences Mall, Vancouver, BC, V6T 1Z3, Canada
    J Bioenerg Biomembr 39:507-17. 2007
    ....
  3. ncbi request reprint Retinoschisin (RS1), the protein encoded by the X-linked retinoschisis gene, is anchored to the surface of retinal photoreceptor and bipolar cells through its interactions with a Na/K ATPase-SARM1 complex
    Laurie L Molday
    Department of Biochemistry and Molecular Biology, Centre for Macular Research, University of British Columbia, Vancouver, British Columbia, Canada
    J Biol Chem 282:32792-801. 2007
    ..This interaction may be part of a novel SARM1-mediated cell signaling pathway required for the maintenance of retinal cell organization and photoreceptor-bipolar synaptic structure...
  4. pmc The role of the photoreceptor ABC transporter ABCA4 in lipid transport and Stargardt macular degeneration
    Robert S Molday
    Department of Biochemistry, Centre for Macular Research, University of British Columbia, Vancouver, B C, Canada V6T 1Z3
    Biochim Biophys Acta 1791:573-83. 2009
    ..Knowledge of the genetic and molecular basis for ABCA4 related retinal degenerative diseases is being used to develop rationale therapeutic treatments for this set of disorders...
  5. ncbi request reprint Focus on molecules: retinoschisin (RS1)
    Robert S Molday
    Department of Biochemistry, Centre for Macular Research, University of British Columbia, Vancouver, BC V6T 1Z3 Canada
    Exp Eye Res 84:227-8. 2007
  6. pmc Defective lipid transport and biosynthesis in recessive and dominant Stargardt macular degeneration
    Robert S Molday
    Department of Biochemistry and Molecular Biology, Centre of Macular Research, University of British Columbia, 2350 Health Sciences Mall, Vancouver, BC, Canada
    Prog Lipid Res 49:476-92. 2010
    ..This review focuses on the molecular characterization of ABCA4 and ELOVL4 and their role in photoreceptor cell biology and the pathogenesis of Stargardt disease...
  7. ncbi request reprint RS1, a discoidin domain-containing retinal cell adhesion protein associated with X-linked retinoschisis, exists as a novel disulfide-linked octamer
    Winco W H Wu
    Department of Biochemistry and Molecular Biology, University of British Columbia, Vancouver, British Columbia, V6T 1Z3, Canada
    J Biol Chem 280:10721-30. 2005
    ..Because mutations that disrupt subunit assembly cause X-linked retinoschisis, the assembly of RS1 into a disulfide-linked homo-octamer appears to be critical for its function as a retinal cell adhesion protein...
  8. ncbi request reprint Disease mechanisms and gene therapy in a mouse model for X-linked retinoschisis
    Laurie L Molday
    University of British Columbia, Vancouver, BC
    Adv Exp Med Biol 572:283-9. 2006
  9. ncbi request reprint Binding of N-retinylidene-PE to ABCA4 and a model for its transport across membranes
    Robert S Molday
    University of British Columbia, Vancouver, BC
    Adv Exp Med Biol 572:465-70. 2006
  10. pmc In vivo imaging of the mouse model of X-linked juvenile retinoschisis with fourier domain optical coherence tomography
    Jing Xu
    School of Engineering Science, Simon Fraser University, Burnaby, British Columbia, Canada
    Invest Ophthalmol Vis Sci 50:2989-93. 2009
    ..The purpose of this study was to investigate Fourier domain optical coherence tomography (FD OCT) as a noninvasive tool for retinal imaging in the Rs1h-knockout mouse (model for X-linked juvenile retinoschisis)...
  11. ncbi request reprint Dominant negative mechanism underlies autosomal dominant Stargardt-like macular dystrophy linked to mutations in ELOVL4
    Celene Grayson
    Department of Biochemistry and Molecular Biology, Centre for Macular Research, University of British Columbia, Vancouver, Canada
    J Biol Chem 280:32521-30. 2005
    ..This dominant negative mechanism contributes to the autosomal dominant inheritance of Stargardt-like macular dystrophy...
  12. ncbi request reprint Defective discoidin domain structure, subunit assembly, and endoplasmic reticulum processing of retinoschisin are primary mechanisms responsible for X-linked retinoschisis
    Winco W H Wu
    Department of Biochemistry and Molecular Biology, University of British Columbia, Vancouver, British Columbia, Canada
    J Biol Chem 278:28139-46. 2003
    ....
  13. ncbi request reprint Secretory carrier membrane proteins interact and regulate trafficking of the organellar (Na+,K+)/H+ exchanger NHE7
    Paulo J C Lin
    Department of Biochemistry and Molecular Biology, The University of British Columbia, 2146 Health Sciences Mall, Vancouver, BC V6T 1Z4, Canada
    J Cell Sci 118:1885-97. 2005
    ..We propose a model wherein SCAMPs participate in the shuttling of NHE7 between recycling vesicles and the TGN...
  14. ncbi request reprint Binding of retinoids to ABCA4, the photoreceptor ABC transporter associated with Stargardt macular degeneration
    Ming Zhong
    Department of Biochemistry and Molecular Biology, University of British Columbia, Vancouver, BC, Canada
    Methods Mol Biol 652:163-76. 2010
    ..These procedures and related radiometric assays using titrated retinal have been used to study the binding of N-retinylidene-PE to wild-type and mutant ABCA4 in the absence and presence of nucleotides for structure-function studies...
  15. ncbi request reprint Regulation of the rod photoreceptor cyclic nucleotide-gated channel
    Rene Warren
    Department of Biochemistry, University of British Columbia, Vancouver, B C Canada V6TIZ3
    Adv Exp Med Biol 514:205-23. 2002
    ..We also show that the beta-subunit of the channel is phosphorylated by endogenous and exogenous casein kinase 2. This posttranslational modification, however, does not alter the sensitivity of the channel for cGMP...
  16. pmc Role of the C terminus of the photoreceptor ABCA4 transporter in protein folding, function, and retinal degenerative diseases
    Ming Zhong
    Department of Biochemistry and Molecular Biology, Centre for Macular Research, University of British Columbia, Vancouver, British Columbia V6T 1Z3, Canada
    J Biol Chem 284:3640-9. 2009
    ..This motif also contributes to the efficient folding of ABCA1 into an active protein. Our results provide a molecular based rationale for the disease phenotype displayed by individuals with mutations in the C terminus of ABCA4...
  17. pmc Proteomics of photoreceptor outer segments identifies a subset of SNARE and Rab proteins implicated in membrane vesicle trafficking and fusion
    Michael C M Kwok
    Department of Biochemistry and Molecular Biology, University of British Columbia, Vancouver, British Columbia V6T 1Z3, Canada
    Mol Cell Proteomics 7:1053-66. 2008
    ..The data set generated in this study is a valuable resource for further analysis of photoreceptor outer segment structure and function...
  18. ncbi request reprint Coexpression and interaction of wild-type and missense RS1 mutants associated with X-linked retinoschisis: its relevance to gene therapy
    Frank M Dyka
    Department of Biochemistry and Molecular Biology, Centre for Macular Research, University of British Columbia, Vancouver, Canada
    Invest Ophthalmol Vis Sci 48:2491-7. 2007
    ..The purpose of this study was to examine the effect that coexpression of wild-type RS1 with disease-causing mutants has on RS1 expression, oligomerization, and secretion to further evaluate gene therapy as a possible treatment for XLRS...
  19. ncbi request reprint Role of subunit assembly in autosomal dominant retinitis pigmentosa linked to mutations in peripherin 2
    Robert S Molday
    Department of Biochemistry and Molecular Biology, Visual Sciences, University of British Columbia, 2146 Health Sciences Mall, Vancouver, BC V6T 1Z3, Canada
    Novartis Found Symp 255:95-112; discussion 113-6, 177-8. 2004
    ..Together, these results indicate that peripherin 2 mutations can cause ADRP either through a deficiency in WT peripherin 2 (C214S, 1.185P) or by a dominant negative effect on disk stability (P216L)...
  20. ncbi request reprint Functional interaction between the two halves of the photoreceptor-specific ATP binding cassette protein ABCR (ABCA4). Evidence for a non-exchangeable ADP in the first nucleotide binding domain
    Jinhi Ahn
    Department of Biochemistry and Molecular Biology, University of British Columbia, Vancouver, British Columbia V6T 1Z3, Canada
    J Biol Chem 278:39600-8. 2003
    ..Our results indicate that only NBD2 of ABCR binds and hydrolyzes ATP in the presence or absence of retinal. NBD1, containing a bound ADP, associates with NBD2 to play a crucial, non-catalytic role in ABCR function...
  21. pmc Localization, purification, and functional reconstitution of the P4-ATPase Atp8a2, a phosphatidylserine flippase in photoreceptor disc membranes
    Jonathan A Coleman
    Department of Biochemistry and Molecular Biology, University of British Columbia, Vancouver, British Columbia V6T 1Z3, Canada
    J Biol Chem 284:32670-9. 2009
    ....
  22. ncbi request reprint N-retinylidene-phosphatidylethanolamine is the preferred retinoid substrate for the photoreceptor-specific ABC transporter ABCA4 (ABCR)
    Seelochan Beharry
    Department of Biochemistry and Molecular Biology, University of British Columbia, Vancouver, British Columbia V6T 1Z3, Canada
    J Biol Chem 279:53972-9. 2004
    ..Our studies further suggest that ATP binding and hydrolysis induces a protein conformational change that causes N-retinylidene-phosphatidylethanolamine to dissociate from ABCA4...
  23. pmc Characterization and purification of the discoidin domain-containing protein retinoschisin and its interaction with galactose
    Frank M Dyka
    Department of Biochemistry and Molecular Biology, Centre for Macular Research, University of British Columbia, 2350 Health Sciences Mall, Vancouver, BC V6T 1Z3, Canada
    Biochemistry 47:9098-106. 2008
    ..This cell expression and protein purification method should prove useful in the isolation of RS1 for detailed structure-function studies...
  24. doi request reprint A carboxy-terminal affinity tag for the purification and mass spectrometric characterization of integral membrane proteins
    Julie P Wong
    Biomedical Research Centre, Department of Biochemistry and Molecular Biology, University of British Columbia, Vancouver, British Columbia, Canada
    J Proteome Res 8:2388-96. 2009
    ..Our results demonstrate that the 1D4-tag enrichment strategy is a versatile tool for the characterization of integral membrane proteins that can be employed for functional proteomic studies...
  25. doi request reprint Effect of late-stage therapy on disease progression in AAV-mediated rescue of photoreceptor cells in the retinoschisin-deficient mouse
    Andreas Janssen
    Institute of Human Genetics, University of Regensburg, Regensburg, Germany
    Mol Ther 16:1010-7. 2008
    ....
  26. pmc Inactivation of the murine X-linked juvenile retinoschisis gene, Rs1h, suggests a role of retinoschisin in retinal cell layer organization and synaptic structure
    Bernhard H F Weber
    Institute of Human Genetics, Biocenter, University of Wurzburg, D 97074 Wurzburg, Germany
    Proc Natl Acad Sci U S A 99:6222-7. 2002
    ....
  27. ncbi request reprint An unusual X-linked retinoschisis phenotype and biochemical characterization of the W112C RS1 mutation
    Alessandro Iannaccone
    Hamilton Eye Institute, Department of Ophthalmology, University of Tennessee Health Science Center, 930 Madison Avenue, Suite 731, Memphis, TN 38163, USA
    Vision Res 46:3845-52. 2006
    ....
  28. ncbi request reprint Prolonged recovery of retinal structure/function after gene therapy in an Rs1h-deficient mouse model of x-linked juvenile retinoschisis
    Seok H Min
    Department of Ophthalmology, University of Florida College of Medicine, Gainesville, 32610, USA
    Mol Ther 12:644-51. 2005
    ....
  29. ncbi request reprint Novel retinal and cone photoreceptor transcripts revealed by human macular expression profiling
    Daniel M Hornan
    Institute of Ophthalmology, Molecular Genetics, University College, London, United Kingdom
    Invest Ophthalmol Vis Sci 48:5388-96. 2007
    ..The macula is essential for visual acuity. It contains many more cone photoreceptors than does the peripheral retina. In this study, macular gene expression was compared with that in the rod-rich peripheral retina...
  30. ncbi request reprint Membrane-associated guanylate kinase proteins MPP4 and MPP5 associate with Veli3 at distinct intercellular junctions of the neurosensory retina
    Heidi Stohr
    Institut fur Humangenetik, Biozentrum, Am Hubland, Universitat Wurzburg, 97074 Wurzburg, Germany
    J Comp Neurol 481:31-41. 2005
    ....
  31. pmc The heteromeric cyclic nucleotide-gated channel adopts a 3A:1B stoichiometry
    Haining Zhong
    Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Nature 420:193-8. 2002
    ..Biochemical analysis of the purified bovine rod CNG channel confirmed this conclusion. This revised stoichiometry provides a new foundation for understanding the structure and function of the CNG channel family...
  32. pmc Identification of photoreceptor genes affected by PRPF31 mutations associated with autosomal dominant retinitis pigmentosa
    Daniel Mordes
    Department of Pediatrics, John F Kennedy Center for Research on Human Development, Vanderbilt University Medical Center, USA
    Neurobiol Dis 26:291-300. 2007
    ..Our results provide an explanation for the photoreceptor-specific phenotype of PRPF31 mutations...

Research Grants30

  1. ROS MEMBRANE PROTEINS AND RETINAL DEGENERATIVE DISEASES
    ROBERT MOLDAY; Fiscal Year: 2000
    ....
  2. ROS MEMBRANE PROTEINS AND RETINAL DEGENERATIVE DISEASES
    ROBERT MOLDAY; Fiscal Year: 2001
    ....
  3. ROS MEMBRANE PROTEINS AND RETINAL DEGENERATIVE DISEASES
    ROBERT MOLDAY; Fiscal Year: 2002
    ....
  4. ROS MEMBRANE PROTEINS AND RETINAL DEGENERATIVE DISEASES
    ROBERT MOLDAY; Fiscal Year: 2003
    ....
  5. ROS MEMBRANE PROTEINS AND RETINAL DEGENERATIVE DISEASES
    ROBERT MOLDAY; Fiscal Year: 2004
    ....
  6. Molecular Basis for Rod Outer Segment Structure and Retinal Degenerative Diseases
    ROBERT MOLDAY; Fiscal Year: 2006
    ....
  7. Molecular Basis for Rod Outer Segment Structure and Retinal Degenerative Diseases
    ROBERT MOLDAY; Fiscal Year: 2007
    ....
  8. Molecular Basis for Rod Outer Segment Structure and Retinal Degenerative Diseases
    ROBERT MOLDAY; Fiscal Year: 2009
    ....
  9. RETINAL PHOTORECEPTOR MEMBRANE PROTEINS
    ROBERT MOLDAY; Fiscal Year: 1980
    ..Regions of the polypeptide chains of these proteins, however, are exposed on the external or cytoplasmic surface of discs and susceptible to degradation by proteases. Thus, rhodopsin and ROS 1.2 appear to be transmembrane glycoproteins. ..
  10. STRUCTURE/FUNCTION OF ROS PLASMA MEMBRANE PROTEINS
    ROBERT MOLDAY; Fiscal Year: 1999
    ....
  11. MOLECULAR PROPERTIES OF ROD PLASMA MEMBRANE PROTEINS
    ROBERT MOLDAY; Fiscal Year: 1990
    ....
  12. MOLECULAR PROPERTIES OF ROD PLASMA MEMBRANE PROTEINS
    ROBERT MOLDAY; Fiscal Year: 1993
    ..The results of this study should provide new insight into the molecular structure and function of important membrane proteins of photoreceptor cells and serve as a basis for defining their role in photoreceptor degenerative diseases...
  13. MOLECULAR PROPERTIES OF ROD PLASMA MEMBRANE PROTEINS
    ROBERT MOLDAY; Fiscal Year: 1991
    ....
  14. MOLECULAR PROPERTIES OF ROD PLASMA MEMBRANE PROTEINS
    ROBERT MOLDAY; Fiscal Year: 1992
    ....
  15. Molecular Basis for Rod Outer Segment Structure and Retinal Degenerative Diseases
    Robert S Molday; Fiscal Year: 2010
    ....