The intraflagellar transport protein, IFT88, is essential for vertebrate photoreceptor assembly and maintenance
Authors
Pazour, Gregory J.Baker, Sheila A.
Deane, James A.
Cole, Douglas G.
Dickert, Bethany L.
Rosenbaum, Joel L.
Witman, George B.
Besharse, Joseph C.
Document Type
Journal ArticlePublication Date
2002-03-28Keywords
AnimalsBlindness
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
Cell Biology
Metadata
Show full item recordAbstract
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.Source
J Cell Biol. 2002 Apr 1;157(1):103-13. Epub 2002 Mar 26. Link to article on publisher's siteDOI
10.1083/jcb.200107108Permanent Link to this Item
http://hdl.handle.net/20.500.14038/42591PubMed ID
11916979Related Resources
Link to Article in PubMedae974a485f413a2113503eed53cd6c53
10.1083/jcb.200107108