|
|
|
|||||||
|
|||||||||
J Biol Chem, Vol. 275, Issue 8, 5370-5378, February 25, 2000
From the Department of Biochemistry and Molecular Biology,
University of British Columbia, Vancouver,
British Columbia V6T 1Z3, Canada
Peripherin/Rds is a tetraspanning membrane
protein that has been implicated in photoreceptor outer segment
morphogenesis and inherited retinal degenerative diseases. Together
with the structurally related protein, Rom-1, it forms a complex along
the rims of rod and cone disc membranes. We have compared the
oligomeric structure of these proteins from nonreduced and
dithiothreitol reduced membranes by velocity sedimentation, SDS-gel
electrophoresis, immunoaffinity chromatography, and chemical
cross-linking. Under reducing conditions peripherin/Rds and Rom-1
existed as homomeric and heteromeric core complexes devoid of
intermolecular disulfide bonds. Under nonreducing conditions core
complexes associated through intermolecular disulfide bonds to form
oligomers. One intermediate-size oligomer contained monomers and
disulfide-linked dimers of peripherin/Rds and Rom-1, while larger
oligomers consisted only of disulfide-linked peripherin/Rds dimers when
analyzed on nonreducing SDS gels. Consistent with this result, disc
membranes contained twice as much peripherin/Rds as Rom-1.
Peripherin/Rds individually expressed in COS-1 cells also formed
disulfide-linked oligomers bridged through Cys-150 residues, whereas
Rom-1 showed little tendency to form oligomers. These results indicate
that peripherin/Rds and Rom-1 associate noncovalently to form
multisubunit core complexes. Peripherin/Rds containing core complexes
interact through specific intermolecular disulfide bonds to form
oligomers which may play a crucial role in photoreceptor disc
morphogenesis and retinal degenerative diseases.
Disulfide-mediated Oligomerization of Peripherin/Rds and
Rom-1 in Photoreceptor Disk Membranes
IMPLICATIONS FOR PHOTORECEPTOR OUTER SEGMENT MORPHOGENESIS AND
DEGENERATION*
and
*
This work was supported in part by the RP Eye Research
Foundation of Canada and the National Eye Institute Grant EY 2422.The costs of publication of this
article were defrayed in part by the
payment of page charges. The article
must therefore be hereby marked
"advertisement" in
accordance with 18 U.S.C. Section
1734 solely to indicate this fact.
Recipient of a predoctoral fellowship from the RP Eye Foundation
of Canada.
§
To whom correspondence should be addressed: Dept. of Biochemistry
and Molecular Biology, 2146 Health Sciences Mall, University of British
Columbia, Vancouver, British Columbia, V6T 1Z3 Canada. Tel.:
604-822-6173; Fax: 604-822-5227; E-mail:
molday@interchange.ubc.ca.
Copyright © 2000 by The American Society for Biochemistry and Molecular Biology, Inc.
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
This article has been cited by other articles:
|
|
 |
|
  K. P. Lim, S. P. Yip, S. C. Cheung, K. W. Leung, S. T. S. Lam, and C. H. To Novel PRPF31 and PRPH2 Mutations and Co-occurrence of PRPF31 and RHO Mutations in Chinese Patients With Retinitis Pigmentosa Arch Ophthalmol, June 1, 2009; 127(6): 784 - 790. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. Chakraborty, X.-Q. Ding, S. M. Conley, S. J. Fliesler, and M. I. Naash Differential requirements for retinal degeneration slow intermolecular disulfide-linked oligomerization in rods versus cones Hum. Mol. Genet., March 1, 2009; 18(5): 797 - 808. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. C. M. Kwok, J. M. Holopainen, L. L. Molday, L. J. Foster, and R. S. Molday Proteomics of Photoreceptor Outer Segments Identifies a Subset of SNARE and Rab Proteins Implicated in Membrane Vesicle Trafficking and Fusion Mol. Cell. Proteomics, June 1, 2008; 7(6): 1053 - 1066. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. F. X. Goldberg, L. M. Ritter, N. Khattree, N. S. Peachey, R. N. Fariss, L. Dang, M. Yu, and A. R. Bottrell An Intramembrane Glutamic Acid Governs Peripherin/rds Function for Photoreceptor Disk Morphogenesis Invest. Ophthalmol. Vis. Sci., July 1, 2007; 48(7): 2975 - 2986. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Nickell, P. S.-H. Park, W. Baumeister, and K. Palczewski Three-dimensional architecture of murine rod outer segments determined by cryoelectron tomography J. Cell Biol., June 21, 2007; 177(5): 917 - 925. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 B P Leroy, A Kailasanathan, J-J De Laey, G C M Black, and F D C Manson Intrafamilial phenotypic variability in families with RDS mutations: exclusion of ROM1 as a genetic modifier for those with retinitis pigmentosa Br. J. Ophthalmol., January 1, 2007; 91(1): 89 - 93. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. S. Lee, B. Burnside, and J. G. Flannery Characterization of Peripherin/rds and Rom-1 Transport in Rod Photoreceptors of Transgenic and Knockout Animals Invest. Ophthalmol. Vis. Sci., May 1, 2006; 47(5): 2150 - 2160. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. Batra-Safferling, K. Abarca-Heidemann, H. G. Korschen, C. Tziatzios, M. Stoldt, I. Budyak, D. Willbold, H. Schwalbe, J. Klein-Seetharaman, and U. B. Kaupp Glutamic Acid-rich Proteins of Rod Photoreceptors Are Natively Unfolded J. Biol. Chem., January 20, 2006; 281(3): 1449 - 1460. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Damek-Poprawa, J. Krouse, C. Gretzula, and K. Boesze-Battaglia A Novel Tetraspanin Fusion Protein, Peripherin-2, Requires a Region Upstream of the Fusion Domain for Activity J. Biol. Chem., March 11, 2005; 280(10): 9217 - 9224. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. M. Ritter, K. Boesze-Battaglia, B. M. Tam, O. L. Moritz, N. Khattree, S.-C. Chen, and A. F. X. Goldberg Uncoupling of Photoreceptor Peripherin/rds Fusogenic Activity from Biosynthesis, Subunit Assembly, and Targeting: A POTENTIAL MECHANISM FOR PATHOGENIC EFFECTS J. Biol. Chem., September 17, 2004; 279(38): 39958 - 39967. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
X.-Q. Ding, M. Nour, L. M. Ritter, A. F.X. Goldberg, S. J. Fliesler, and M. I. Naash The R172W mutation in peripherin/rds causes a cone-rod dystrophy in transgenic mice Hum. Mol. Genet., September 15, 2004; 13(18): 2075 - 2087. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Nour, X.-Q. Ding, H. Stricker, S. J. Fliesler, and M. I. Naash Modulating Expression of Peripherin/rds in Transgenic Mice: Critical Levels and the Effect of Overexpression Invest. Ophthalmol. Vis. Sci., August 1, 2004; 45(8): 2514 - 2521. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. M. Toler Oxidative Stress Plays an Important Role in the Pathogenesis of Drug-Induced Retinopathy Experimental Biology and Medicine, July 1, 2004; 229(7): 607 - 615. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 B. M. Tam, O. L. Moritz, and D. S. Papermaster The C Terminus of Peripherin/rds Participates in Rod Outer Segment Targeting and Alignment of Disk Incisures Mol. Biol. Cell, April 1, 2004; 15(4): 2027 - 2037. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. J.R. Loewen, O. L. Moritz, B. M. Tam, D. S. Papermaster, and R. S. Molday The Role of Subunit Assembly in Peripherin-2 Targeting to Rod Photoreceptor Disk Membranes and Retinitis Pigmentosa Mol. Biol. Cell, August 1, 2003; 14(8): 3400 - 3413. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Li, X.-Q. Ding, J. O'Brien, M. R. Al-Ubaidi, and M. I. Naash Molecular Characterization of the Skate Peripherin/rds Gene: Relationship to Its Orthologues and Paralogues Invest. Ophthalmol. Vis. Sci., June 1, 2003; 44(6): 2433 - 2441. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. S.-T. Kai, G. S. Butler, C. J. Morrison, A. E. King, G. R. Pelman, and C. M. Overall Utilization of a Novel Recombinant Myoglobin Fusion Protein Expression System to Characterize the Tissue Inhibitor of Metalloproteinase (TIMP)-4 and TIMP-2 C-terminal Domain and Tails by Mutagenesis. THE IMPORTANCE OF ACIDIC RESIDUES IN BINDING THE MMP-2 HEMOPEXIN C DOMAIN J. Biol. Chem., December 6, 2002; 277(50): 48696 - 48707. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. Boesze-Battaglia, J. Dispoto, and M. A. Kahoe Association of a Photoreceptor-specific Tetraspanin Protein, ROM-1, with Triton X-100-resistant Membrane Rafts from Rod Outer Segment Disk Membranes J. Biol. Chem., October 25, 2002; 277(44): 41843 - 41849. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Poetsch, L. L. Molday, and R. S. Molday 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., December 14, 2001; 276(51): 48009 - 48016. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. F. X. Goldberg, L. M. Fales, J. B. Hurley, and N. Khattree Folding and Subunit Assembly of Photoreceptor Peripherin/rds Is Mediated by Determinants within the Extracellular/Intradiskal EC2 Domain. IMPLICATIONS FOR HETEROGENEOUS MOLECULAR PATHOLOGIES J. Biol. Chem., November 9, 2001; 276(46): 42700 - 42706. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W. Kedzierski, S. Nusinowitz, D. Birch, G. Clarke, R. R. McInnes, D. Bok, and G. H. Travis Deficiency of rds/peripherin causes photoreceptor death in mouse models of digenic and dominant retinitis pigmentosa PNAS, June 20, 2001; (2001) 141124198. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. J. R. Loewen, O. L. Moritz, and R. S. Molday Molecular Characterization of Peripherin-2 and Rom-1 Mutants Responsible for Digenic Retinitis Pigmentosa J. Biol. Chem., June 15, 2001; 276(25): 22388 - 22396. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Bungert, L. L. Molday, and R. S. Molday Membrane Topology of the ATP Binding Cassette Transporter ABCR and Its Relationship to ABC1 and Related ABCA Transporters. IDENTIFICATION OF N-LINKED GLYCOSYLATION SITES J. Biol. Chem., June 22, 2001; 276(26): 23539 - 23546. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
W. Kedzierski, S. Nusinowitz, D. Birch, G. Clarke, R. R. McInnes, D. Bok, and G. H. Travis Deficiency of rds/peripherin causes photoreceptor death in mouse models of digenic and dominant retinitis pigmentosa PNAS, July 3, 2001; 98(14): 7718 - 7723. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. E. Hemler Specific tetraspanin functions J. Cell Biol., December 24, 2001; 155(7): 1103 - 1108. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| All ASBMB Journals | Molecular and Cellular Proteomics |
| Journal of Lipid Research | ASBMB Today |