Molday Lab Publications

Selected Publications

Wu, WW and Molday RS. (2003). Defective discoidin domain structure, subunit assembly, and endoplasmic reticulum processing of retinoschisin are primary mechanisms responsible for X-linked retinoschisis. J Biol Chem 278:28139-46.

Loewen, CJ, Moritz OL, Tam BM, Papermaster DS, and Molday RS. (2003). The role of subunit assembly in peripherin-2 targeting to rod photoreceptor disk membranes and retinitis pigmentosa Mol Biol Cell. 14:3400-13.

Ahn J., Beharry, S., Molday, L.L. and Molday, R.S. (2003). 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. J. Biol. Chem., 278:39600-39608.

Weber, B.H.F., Schrewe, H., Molday, L.L., Gehrig, A., White, K., Seeliger, M.W, Jaissle, G.B., Friedburg, C., Tamm, T., and Molday, R.S. (2002) Inactivation of the murine X-linked juvenile retinoschisis gene, Rs1h, suggests a role of retinoschisin in retinal cell layer organization and synaptic structure. Proc. Natl. Acad. Sci. U.S.A. 99:6222-6227.

Zhong, H., Molday, L.L., Molday, R.S. and Yau, K-W. (2002) The Heteromeric Cyclic Nucleotide-gated Channel Adopts a 3A:1B stoichiometry. Nature 420:193-198.

Warren, R. and Molday RS. (2002) Regulation of the rod photoreceptor cyclic nucleotide-gated channel. Adv Exp Med Biol. 514:205-23.

Poetsch, A., Molday, L.L. and Molday, R.S. (2001) The cGMP-gated Channel and Related Glutamic Acid Rich Proteins Interact with Peripherin-2 at the Rim Region of Rod Photoreceptor Disc Membranes. J. Biol. Chem. 276:48009-48016.

Bungert, S., Molday, L.L. and Molday, R.S. (2001) Membrane Topology of ABCR (ABCA4), ABC1 (ABCA1), and Related ABCA Transporters. J. Biol. Chem. 276:23539-23546

Loewen, C.J.R. and Molday, R.S. (2001) Molecular Characterization of Peripherin-2 and Rom-1 Mutants Responsible for Digneic Retinitis Pigmentosa. J. Biol. Chem. 276:22388-22396.

Molday, L.L., Sauer, C.G., Weber, B.H.F., and Molday, R.S. (2001) Molecular Characterization and Localization of RS1, the Protein Encoded by the Gene for X-linked Juvenile Retinoschisis. Invest. Ophthalmol. Vis. Sci. 42:816-825.

Molday, L.L., Rabin, A.R., and Molday, R.S. (2000) ABCR Expression in Foveal Cone photoreceptors and Its Role in Stargardt Macular Dystrophy. Nature Genetics 25:257-258.

Clarke, G., Goldberg, A.F.X., Vidgen, D., Collins, L., Ploder, L., Schwarz, L., Molday, L.L., Rossant, J., SzÈl, A., Molday, R.S., Birch, D.G. and McInnes, R.R. (2000) Rom-1 is required for rod photoreceptor viability and the regulation of disk morphogenesis. Nature Genetics 25:67-73.

Loewen, C.J.R. and Molday, R.S. (2000) Disulfide-linked Oligomerization of Peripherin/rds and Rom-1 of Photoreceptor Disk Membranes. Implications for Photoreceptor Outer Segment Morphogenesis and Degeneration. J. Biol. Chem. 275:5370-5378.

Ahn, J., Wong, J.T., Molday, R.S. (2000) The Effect of Lipid Environment and Retinoids on the ATPase Activity of ABCR, the Photoreceptor ABC Transporter Responsible for Stargardt Macular Dystrophy. J. Biol. Chem. 275: 20399-20405.42.

Sun, H., Molday, R.S. and Nathans, J. (1999) Retinal Stimulates ATP Hydrolysis by Purified and Reconstituted ABCR, the photoreceptor-specific ABC transporter responsible for Stargardt Disease. J.Biol. Chem. 274:8269-8281.

Goldberg, A.F.X.., Loewen, C.J., and Molday, R.S. (1998) Cysteine Residues of Photoreeceptor Peripherin/rds: Role in Subunit Assembly and Autosomoal Dominant Retinitis Pigmentosa. Biochemistry 37, 680-685.

Nasonkin, I., Illing, M., Koehler, M, Schmid, M., Molday, R.S. and Weber, B.H.F. (1998) Mapping of the Rod Photoreceptor ABC Transporter (ABCR) to 1p21-p22.1 and identification of novel mutations in Stargardt’s Disease. Human Genetics 102, 21-26.

Molday, R.S. and Molday, L.L. (1998) The Molecular Properties of the cGMP-gated Channel of Rod Photoreceptors. Vision Res. 38: 1315-13232.

Kim, T.-Y., Reid, D.M., and Molday, R.S. (1998) Structure-Function Relationships and Localization of the Na/Ca-K Exchanger in Rod Photoreceptors. J. Biol. Chem. 273: 16561-16567.

Molday, R.S. (1998) Photoreceptor Outer Segment Proteins, Phototransduction and Retinal Degenerative Diseases: The Friedenwald Lecture. Invest. Ophthal. Vis. Sci. 39: 2493-2509.

Schwarzer, A., Kim, T., Hagen, V., Molday, R.S. and Bauer, P.J. (1997) The Na/Ca-K Exchanger of Rod Photoreceptors exists as a Dimer in the Plasma Membrane. Biochemistry 36, 13667-13676.

Illing, M., Molday, L.L. and Molday, R.S. (1997) The Rim Protein of Retinal Rod Outer Segments is a Member of the ABC Transporter Superfamily. J. Biol. Chem. 272: 10303-10310.

Colville, C. and Molday, R.S. (1996) Primary Structure and Expression of the Human beta-subunit and Related Proteins of the Rod Photoreceptor Cyclic Nucleotide Gated Channel. J. Biol. Chem. 271, 32968-32974.

Goldberg, A.F.X. and Molday, R.S. (1996) Defective Subunit Assembly Underlies a Novel Digenic Form of Retinitis Pigmentosa Linked to Mutations in Peripherin/rds and Rom-1. Proc. Natl. Acad. Sci. USA 93, 13726-13730.

Moritz, O.L. and Molday, R.S. (1996) Molecular Cloning, Membrane Topology and Localization of Bovine Rom-1 in Rod and Cone Photoreceptor Cells. Invest. Ophthal. Vis. Sci. 37: 352-362.

Goldberg, A.F.X. and Molday, R.S. (1996) Subunit Composition of the Peripherin/rds-rom-1 Disk Rim Complex from Rod Photoreceptors: Hydrodynamic Evidence for a Tetrameric Quaternary Structure. Biochemistry 35: 6144-6149.

Molday, R.S. (1996) Calmodulin Regulation of cGMP-gated Channels. Curr. Opin. Neurobiol. 6, 445-452.

Körschen, H.G., Illing, M., Seifert, R., Sesti, F., Williams, A., Gotzes, S., Colville, C., M¸ller, f., Dose, A., Godde, M., Molday, L., Kaupp, U.B., and Molday, R.S. (1995) A 240 kDa Protein Represents the Complete beta Subunit of the Cyclic Nucleotide-gated Channel from Rod Photoreceptor. Neuron 15, 627-636.

Chen, T.-Y., Illing, M., Molday, L.L., Hsu, Y.-T., Yau, K.-W., and Molday, R.S. (1994) Subunit 2 (or beta) of Retinal Rod cGMP-Gated Cation Channel is a Component of the 240 kD Channel-Associated Protein and Mediates Ca2+-Calmodulin Modulation. Proc. Natl Acad. Sci. U.S.A. 91, 11757-11761.

Hsu, S.-C. and Molday, R.S (1994) Glucose metabolism in photoreceptor outer segments: Its involvement in the maintenance of phototransduction and NADPH-requiring processes. J. Biol. Chem. 269, 17954-17959.

Hsu, Y.-T. and Molday, R.S. (1994) Interaction of Calmodulin with the Cyclic GMP-gated Channel of Rod Photoreceptor Cells: Modulation of Activity, Affinity Purification and Localization. J. Biol. Chem. 269, 29765-29770.

Hsu, Y.-T. and Molday, R.S. (1993) Modulation of the cGMP-gated Channel by Calmodulin. Nature 361, 76-79.

Bönigk, W., Altenhofen, W., Muller, F., Dose, A., Illing, M., Molday, R.S. and Kaupp, U.B. (1993) Rod and cone photoreceptor Cells express distinct genes for cyclic GMP-gated channels. Neuron 10, 865-877.

Bascom, R.A., Manara, S., Collins, L., Molday, R.S., Kalnins, V.I. and McInnes, R.R. (1992) Molecular Cloning of the cDNA for a Novel Photoreceptor-specific Membrane Protein (rom-1) Identifies a Disk Rim Protein Family Implicated in Human Degenerative Retinopathies. Neuron 8, 1171-1184.

Olsson, J.E., Gordon, J.W., Pawlyk, B.S., Roof, D., Hayes, A., Molday, R.S., Mukai, S., Cowley, G.S., Berson, E.L. and Dryja, T.P. (1992) Retinal Degeneration in Transgenic mice with a mutant or Wild-type Copy of the Human Rhodopsin Gene. Neuron 9, 815-830.

Arikawa, K., Molday, L.L., Molday, R.S. and Williams, D.S. (1992) Localization of Peripherin/rds in the Disk Membranes of Cone and Rod Photoreceptors: Relationship to Disk Membrane Morphogenesis and Retinal Degeneration. J. Cell Biol. 116, 659-667.

Hsu, S-C. and Molday, R.S. (1991) Glycolytic Enzymes and a GLUT-1 Glucose Transporter in the Outer Segments of Rod and Cone Photoreceptor Cells. J. Biol. Chem. 266, 21745-21752.

Molday, R.S., Molday, L.L., Dose, A., Clark-Lewis, I., Illing, M., Cook, N.J., Eismann, E. and Kaupp, U.B. (1991) The cGMP-gated Channel of the Rod Photoreceptor Cell. Characterization and Orientation of the N-terminus. J. Biol. Chem 266, 21917-21922.

Connell, G., Bascom, R., Molday, L., Reid, D., McInnes, R.R. and Molday, R.S. (1991) Photoreceptor Peripherin is the Normal Product of the Gene Responsible for Retinal Degeneration in the rds Mouse. Proc. Natl. Acad. Sci. USA 88, 723-726.

Connell, G. and Molday, R.S. (1990) Molecular Cloning, Primary Structure, and Orientation of the Vertebrate Photoreceptor Cell Protein Peripherin in the Rod Outer Segment Disk Membrane. Biochemistry 29, 4691-4698.

Hsu, S-C. and Molday, R.S. (1990) Glyceraldehyde 3 Phosphate Dehydrogenase is a Major Protein Associated with the Plasma Membrane of Retinal Photoreceptor Outer Segments. J. Biol. Chem. 265, 13308-13313.

Doi, T., Molday, R.S. and Khorana, H.G. (1990) The Role of the Intradiscal Domain in Rhodopsin Assembly and Function. Proc. Natl. Acad. Sci. 87, 4991-4995.

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