The intraflagellar transport protein, IFT88, is essential for vertebrate photoreceptor assembly and maintenance. J Cell Biol 2002 Apr 01;157(1):103-13
Date
03/28/2002Pubmed ID
11916979Pubmed Central ID
PMC2173265DOI
10.1083/jcb.200107108Scopus ID
2-s2.0-0036544554 (requires institutional sign-in at Scopus site) 395 CitationsAbstract
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.
Author List
Pazour GJ, Baker SA, Deane JA, Cole DG, Dickert BL, Rosenbaum JL, Witman GB, Besharse JCMESH terms used to index this publication - Major topics in bold
AnimalsBlindness
Cattle
Chlamydomonas
Cilia
Flagella
Male
Mice
Mice, Mutant Strains
Molecular Sequence Data
Mutagenesis, Insertional
Plant Proteins
Proteins
Protozoan Proteins
Rabbits
Retinal Rod Photoreceptor Cells
Rod Opsins
Testis
Transport Vesicles
Tumor Suppressor Proteins
