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PRPH2  -  peripherin 2 (retinal degeneration, slow)

Bos taurus

 
 
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Disease relevance of RDS

  • This work describes novel roles for the C terminus of peripherin/rds in targeting and maintaining ROS structure and its potential involvement in inherited retinal degenerations [1].
 

High impact information on RDS

  • A 220-kD concanavalin A-binding glycoprotein and peripherin, a rim-specific protein, were also present along with minor proteins of 43 and 57-63 kD [2].
  • They may provide flexible spacers or linkers tethering the cyclic GMP-gated channel in the plasma membrane to peripherin at the disc rim to produce a stack of rings of these protein complexes along the long axis of the outer segment [3].
  • The C terminus of peripherin/rds participates in rod outer segment targeting and alignment of disk incisures [1].
  • A GFP fusion containing residues 317-336 of peripherin/rds localized uniformly to disk membranes [1].
  • Peripherin/retinal degeneration slow (rds) and rom-1 are photoreceptor-specific members of the transmembrane 4 superfamily of transmembrane proteins, which participate in disk morphogenesis and localize to rod outer segment (ROS) disk rims [1].
 

Biological context of RDS

 

Anatomical context of RDS

 

Associations of RDS with chemical compounds

 

Other interactions of RDS

  • These results are consistent with the view that the lamellar region of discs containing rhodopsin is a distinct membrane domain from the rim region which contains peripherin, a high Mr rim protein and possibly other proteins involved in disc-disc and disc-plasma membrane interactions [11].
 

Analytical, diagnostic and therapeutic context of RDS

  • A C-terminal CNBr fragment of peripherin was purified by immunoaffinity chromatography and reverse-phase high-performance liquid chromatography [4].
  • The rate of intubation, severity of RDS, rate of surfactant administration, pulmonary airleaks and days on the ventilator did not differ between both groups [12].
  • Western blotting and immunoprecipitation studies showed that the FLAG-tagged bovine peripherin/rds (full-length) was expressed as a 76kDa dimer, which associates with HA-tagged rom-1 to form a higher order complex [7].

References

  1. The C terminus of peripherin/rds participates in rod outer segment targeting and alignment of disk incisures. Tam, B.M., Moritz, O.L., Papermaster, D.S. Mol. Biol. Cell (2004) [Pubmed]
  2. Differences in the protein composition of bovine retinal rod outer segment disk and plasma membranes isolated by a ricin-gold-dextran density perturbation method. Molday, R.S., Molday, L.L. J. Cell Biol. (1987) [Pubmed]
  3. Glutamic acid-rich proteins of rod photoreceptors are natively unfolded. Batra-Safferling, R., Abarca-Heidemann, K., Körschen, H.G., Tziatzios, C., Stoldt, M., Budyak, I., Willbold, D., Schwalbe, H., Klein-Seetharaman, J., Kaupp, U.B. J. Biol. Chem. (2006) [Pubmed]
  4. Molecular cloning, primary structure, and orientation of the vertebrate photoreceptor cell protein peripherin in the rod outer segment disk membrane. Connell, G.J., Molday, R.S. Biochemistry (1990) [Pubmed]
  5. Fusion between retinal rod outer segment membranes and model membranes: a role for photoreceptor peripherin/rds. Boesze-Battaglia, K., Lamba, O.P., Napoli, A.A., Sinha, S., Guo, Y. Biochemistry (1998) [Pubmed]
  6. Purification and light-dependent phosphorylation of a candidate fusion protein, the photoreceptor cell peripherin/rds. Boesze-Battaglia, K., Kong, F., Lamba, O.P., Stefano, F.P., Williams, D.S. Biochemistry (1997) [Pubmed]
  7. Deletional analysis of the rod photoreceptor cell peripherin/RDS carboxy-terminal region. Muller-Weeks, S., Boesze-Battaglia, K., Fitzgerald, C. Exp. Eye Res. (2002) [Pubmed]
  8. Association of a photoreceptor-specific tetraspanin protein, ROM-1, with triton X-100-resistant membrane rafts from rod outer segment disk membranes. Boesze-Battaglia, K., Dispoto, J., Kahoe, M.A. J. Biol. Chem. (2002) [Pubmed]
  9. Cysteine residues of photoreceptor peripherin/rds: role in subunit assembly and autosomal dominant retinitis pigmentosa. Goldberg, A.F., Loewen, C.J., Molday, R.S. Biochemistry (1998) [Pubmed]
  10. ABCR, the ATP-binding cassette transporter responsible for Stargardt macular dystrophy, is an efficient target of all-trans-retinal-mediated photooxidative damage in vitro. Implications for retinal disease. Sun, H., Nathans, J. J. Biol. Chem. (2001) [Pubmed]
  11. Peripherin. A rim-specific membrane protein of rod outer segment discs. Molday, R.S., Hicks, D., Molday, L. Invest. Ophthalmol. Vis. Sci. (1987) [Pubmed]
  12. Neonatal outcome in small for gestational age infants: do they really better? Gortner, L., Wauer, R.R., Stock, G.J., Reiter, H.L., Reiss, I., Jorch, G., Hentschel, R., Hieronimi, G. Journal of perinatal medicine. (1999) [Pubmed]
 
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