Mislocalization of Oligomerization-Incompetent RDS is Associated with Mislocalization of Cone Opsins and Cone Transducin

  • Shannon M. Conley
  • Dibyendu Chakraborty
  • Muna I. NaashEmail author
Conference paper
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 723)


RDS (retinal degeneration slow) is a structural protein found in the disk rims of rod and cone photoreceptor outer segments (OSs). Higher-order oligomeric RDS complexes held together by intramolecular disulfide bonds are required for the formation and maintenance of the rim region. Expression of transgenic RDS which cannot form higher-order oligomers (C150S-RDS) leads to dominant, structural, and functional cone degeneration. Furthermore, in this model, C150S-transgenic protein and opsins are mislocalized throughout the cell in cones but not in rods. The purpose of this study was to determine whether other cone OS proteins are mislocalized in the presence of C150S-RDS. Our results demonstrate that all tested proteins are properly localized in the nrl −/− background. In contrast, in the WT background, cone transducin is mislocalized with M- and S-opsin. These results support the hypothesis that C150S-RDS is interfering with the normal transport of cone opsin vesicles.


Retinitis Pigmentosa Outer Segment Retinal Degeneration Cone Opsin Cyclic Nucleotide Gated Channel 
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  1. Abd-El-Barr MM, Sykoudis K, Andrabi S et al (2007) Impaired photoreceptor protein transport and synaptic transmission in a mouse model of Bardet-Biedl syndrome. Vision Res 47:3394–3407PubMedCrossRefGoogle Scholar
  2. Avasthi P, Watt CB, Williams DS et al (2009) Trafficking of membrane proteins to cone but not rod outer segments is dependent on heterotrimeric kinesin-II. J Neurosci 29:14287–14298PubMedCrossRefGoogle Scholar
  3. Chakraborty D, Ding XQ, Fliesler SJ et al (2008) Outer segment oligomerization of rds: evidence from mouse models and subcellular fractionation. Biochemistry 47:1144–1156PubMedCrossRefGoogle Scholar
  4. Chakraborty D, Conley SM, Stuck MW et al (2010) Differences in RDS trafficking, assembly, and function in cones vs. rods: Insights from studies of C150S-RDS. Hum Mol Genet In PressGoogle Scholar
  5. Chakraborty D, Ding XQ, Conley SM et al (2009) Differential requirements for retinal degeneration slow intermolecular disulfide-linked oligomerization in rods versus cones. Hum Mol Genet 18:797–808PubMedGoogle Scholar
  6. Daniele LL, Lillo C, Lyubarsky AL et al (2005) Cone-like Morphological, Molecular, and Electrophysiological Features of the Photoreceptors of the Nrl Knockout Mouse. Invest Ophthalmol Vis Sci 46:2156–2167PubMedCrossRefGoogle Scholar
  7. Fariss RN, Molday RS, Fisher SK et al (1997) Evidence from normal and degenerating photoreceptors that two outer segment integral membrane proteins have separate transport pathways. J Comp Neurol 387:148–156PubMedCrossRefGoogle Scholar
  8. Farjo R, Skaggs JS, Nagel BA et al (2006) Retention of function without normal disc morphogenesis occurs in cone but not rod photoreceptors. J Cell Biol 173:59–68PubMedCrossRefGoogle Scholar
  9. Karan S, Zhang H, Li S et al (2008) A model for transport of membrane-associated phototransduction polypeptides in rod and cone photoreceptor inner segments. Vision Res 48:442–452PubMedCrossRefGoogle Scholar
  10. Mears AJ, Kondo M, Swain PK et al (2001) Nrl is required for rod photoreceptor development. Nat Genet 29:447–452PubMedCrossRefGoogle Scholar
  11. Sanyal S, Jansen HG (1981) Absence of receptor outer segments in the retina of rds mutant mice. Neurosci Lett 21:23–26PubMedCrossRefGoogle Scholar
  12. Zhu X, Li A, Brown B et al (2002) Mouse cone arrestin expression pattern: light induced translocation in cone photoreceptors. Mol Vis 8:462–471PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Shannon M. Conley
    • 1
  • Dibyendu Chakraborty
    • 1
  • Muna I. Naash
    • 1
    Email author
  1. 1.Department of Cell BiologyUniversity of Oklahoma Health Sciences CenterOklahoma CityUSA

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