Journal
JOURNAL OF CELL BIOLOGY
Volume 157, Issue 1, Pages 103-113Publisher
ROCKEFELLER UNIV PRESS
DOI: 10.1083/jcb.200107108
Keywords
rods and cones; retinitis pigmentosa; blindness; primary cilia; orpk
Categories
Funding
- NEI NIH HHS [R01 EY003222, T32 EY014537, EY-03222, P30 EY001931, EY01931] Funding Source: Medline
- NIDDK NIH HHS [DK 32520, P30 DK032520] Funding Source: Medline
- NIGMS NIH HHS [GM-30626, R37 GM014642, R37 GM030626, R01 GM060992, R01 GM030626, GM-60992, GM-14642, R01 GM014642] Funding Source: Medline
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Approximately 10% of the photoreceptor outer segment (CS) 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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