Department of Cell Biology; Program in Molecular Medicine
Animals; Blindness; Cattle; Chlamydomonas; Cilia; Flagella; Male; Mice; Mice, Mutant Strains; Molecular Sequence Data; Mutagenesis, Insertional; Opsin; Proteins; Protozoan Proteins; Rabbits; Rods (Retina); Testis; Transport Vesicles; *Tumor Suppressor Proteins
Approximately 10% of the photoreceptor outer segment (OS) is turned over each day, requiring large amounts of lipid and protein to be moved from the inner segment to the OS. Defects in intraphotoreceptor transport can lead to retinal degeneration and blindness. The transport mechanisms are unknown, but because the OS is a modified cilium, intraflagellar transport (IFT) is a candidate mechanism. IFT involves movement of large protein complexes along ciliary microtubules and is required for assembly and maintenance of cilia. We show that IFT particle proteins are localized to photoreceptor connecting cilia. We further find that mice with a mutation in the IFT particle protein gene, Tg737/IFT88, have abnormal OS development and retinal degeneration. Thus, IFT is important for assembly and maintenance of the vertebrate OS.
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Citation: J Cell Biol. 2002 Apr 1;157(1):103-13. Epub 2002 Mar 26. Link to article on publisher's site
DOI of Published Version
The Journal of cell biology
Pazour, Gregory J.; Baker, Sheila A.; Deane, James A.; Cole, Douglas G.; Dickert, Bethany L.; Rosenbaum, Joel L.; Witman, George B.; and Besharse, Joseph C., "The intraflagellar transport protein, IFT88, is essential for vertebrate photoreceptor assembly and maintenance" (2002). Open Access Articles. 925.