Research Topics
| ROBERT MOLDAYSummaryAffiliation: University of British Columbia Country: Canada Publications
Research Grants
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Detail Information
Publications
Subretinal gene delivery using helper-dependent adenoviral vectorsLinda 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...
ATP-binding cassette transporter ABCA4: molecular properties and role in vision and macular degenerationRobert 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....- 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 complexLaurie 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... - The role of the photoreceptor ABC transporter ABCA4 in lipid transport and Stargardt macular degenerationRobert 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... - 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 - Defective lipid transport and biosynthesis in recessive and dominant Stargardt macular degenerationRobert 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... - RS1, a discoidin domain-containing retinal cell adhesion protein associated with X-linked retinoschisis, exists as a novel disulfide-linked octamerWinco 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... - Disease mechanisms and gene therapy in a mouse model for X-linked retinoschisisLaurie L Molday
University of British Columbia, Vancouver, BC
Adv Exp Med Biol 572:283-9. 2006 - Binding of N-retinylidene-PE to ABCA4 and a model for its transport across membranesRobert S Molday
University of British Columbia, Vancouver, BC
Adv Exp Med Biol 572:465-70. 2006 - In vivo imaging of the mouse model of X-linked juvenile retinoschisis with fourier domain optical coherence tomographyJing 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)... - Dominant negative mechanism underlies autosomal dominant Stargardt-like macular dystrophy linked to mutations in ELOVL4Celene 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... - Defective discoidin domain structure, subunit assembly, and endoplasmic reticulum processing of retinoschisin are primary mechanisms responsible for X-linked retinoschisisWinco W H Wu
Department of Biochemistry and Molecular Biology, University of British Columbia, Vancouver, British Columbia, Canada
J Biol Chem 278:28139-46. 2003.... - Secretory carrier membrane proteins interact and regulate trafficking of the organellar (Na+,K+)/H+ exchanger NHE7Paulo 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... 
Binding of retinoids to ABCA4, the photoreceptor ABC transporter associated with Stargardt macular degenerationMing 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...- Regulation of the rod photoreceptor cyclic nucleotide-gated channelRene 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... - Role of the C terminus of the photoreceptor ABCA4 transporter in protein folding, function, and retinal degenerative diseasesMing 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... - Proteomics of photoreceptor outer segments identifies a subset of SNARE and Rab proteins implicated in membrane vesicle trafficking and fusionMichael 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... - Coexpression and interaction of wild-type and missense RS1 mutants associated with X-linked retinoschisis: its relevance to gene therapyFrank 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... - Role of subunit assembly in autosomal dominant retinitis pigmentosa linked to mutations in peripherin 2Robert 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)... - 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 domainJinhi 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... - Localization, purification, and functional reconstitution of the P4-ATPase Atp8a2, a phosphatidylserine flippase in photoreceptor disc membranesJonathan 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.... - 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... - Characterization and purification of the discoidin domain-containing protein retinoschisin and its interaction with galactoseFrank 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... - A carboxy-terminal affinity tag for the purification and mass spectrometric characterization of integral membrane proteinsJulie 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... - Effect of late-stage therapy on disease progression in AAV-mediated rescue of photoreceptor cells in the retinoschisin-deficient mouseAndreas Janssen
Institute of Human Genetics, University of Regensburg, Regensburg, Germany
Mol Ther 16:1010-7. 2008.... - Inactivation of the murine X-linked juvenile retinoschisis gene, Rs1h, suggests a role of retinoschisin in retinal cell layer organization and synaptic structureBernhard 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.... - An unusual X-linked retinoschisis phenotype and biochemical characterization of the W112C RS1 mutationAlessandro 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.... - Prolonged recovery of retinal structure/function after gene therapy in an Rs1h-deficient mouse model of x-linked juvenile retinoschisisSeok H Min
Department of Ophthalmology, University of Florida College of Medicine, Gainesville, 32610, USA
Mol Ther 12:644-51. 2005.... - Novel retinal and cone photoreceptor transcripts revealed by human macular expression profilingDaniel 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... - Membrane-associated guanylate kinase proteins MPP4 and MPP5 associate with Veli3 at distinct intercellular junctions of the neurosensory retinaHeidi Stohr
Institut fur Humangenetik, Biozentrum, Am Hubland, Universitat Wurzburg, 97074 Wurzburg, Germany
J Comp Neurol 481:31-41. 2005.... - The heteromeric cyclic nucleotide-gated channel adopts a 3A:1B stoichiometryHaining 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... - Identification of photoreceptor genes affected by PRPF31 mutations associated with autosomal dominant retinitis pigmentosaDaniel 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 Grants
- ROS MEMBRANE PROTEINS AND RETINAL DEGENERATIVE DISEASESROBERT MOLDAY; Fiscal Year: 2004....
- Molecular Basis for Rod Outer Segment Structure and Retinal Degenerative DiseasesROBERT MOLDAY; Fiscal Year: 2007....
- Molecular Basis for Rod Outer Segment Structure and Retinal Degenerative DiseasesROBERT MOLDAY; Fiscal Year: 2009....
- RETINAL PHOTORECEPTOR MEMBRANE PROTEINSROBERT 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. ..
- STRUCTURE/FUNCTION OF ROS PLASMA MEMBRANE PROTEINSROBERT MOLDAY; Fiscal Year: 1999....
- MOLECULAR PROPERTIES OF ROD PLASMA MEMBRANE PROTEINSROBERT 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...
- MOLECULAR PROPERTIES OF ROD PLASMA MEMBRANE PROTEINSROBERT MOLDAY; Fiscal Year: 1992....
- Molecular Basis for Rod Outer Segment Structure and Retinal Degenerative DiseasesRobert S Molday; Fiscal Year: 2010....