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Peripherin/Rds in Skate Retina

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Retinal Degenerations

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 533))

Abstract

Peripherin/rds, a transmembrane glycoprotein, has been localized to the rim region of mature disks and the basal region adjacent to the cilia of rod and cone outer segments where disk morphogenesis occurs (Arikawaet al.1992). The importance of peripherin/rds to photoreceptor outer segment structure is inferred by its involvement in theretinal degeneration slow (rds) mutant mouse line (VanNie et al.1978). Outer segment membrane biogenesis does occur in the homozygousrds mutant, but the membrane is unable to fold into the proper disk structure, suggesting an important role for peripherin/rds in the assembly, orientation, and physical stability of the disks (Travis and Bok, 1993; Molday, 1994). The peripherin/rds cDNA has been isolated and sequenced from human (Traviset al.1991), cow (Connell and Molday, 1990), dog (Moghrabiet al.,1995), cat (Gorinet al.1993), rat (Begy and Bridges, 1990), mouse (Traviset al.,1989), chicken (Wenget al.1998), andXenopus (Kedzierskiet al.1996) and found to code for proteins containing 346 to 364 residues, depending on the species. Studies have shown a noncovalent association between peripherin/rds and its homolog ROM-1 to form homo and heterotetramer and higher oligomers (Goldberg and Molday, 1996).

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© 2003 Springer Science+Business Media New York

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Naash, M.I., Ding, XQ., Li, C., O’Brien, J., Al-Ubaidi, M.R. (2003). Peripherin/Rds in Skate Retina. In: LaVail, M.M., Hollyfield, J.G., Anderson, R.E. (eds) Retinal Degenerations. Advances in Experimental Medicine and Biology, vol 533. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-0067-4_48

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  • DOI: https://doi.org/10.1007/978-1-4615-0067-4_48

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4613-4909-9

  • Online ISBN: 978-1-4615-0067-4

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