UMMS Affiliation

Department of Cell Biology; Program in Molecular Medicine

Date

3-28-2002

Document Type

Article

Subjects

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

Disciplines

Cell Biology

Abstract

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.

Rights and Permissions

Citation: J Cell Biol. 2002 Apr 1;157(1):103-13. Epub 2002 Mar 26. Link to article on publisher's site

Related Resources

Link to Article in PubMed

PubMed ID

11916979

Included in

Cell Biology Commons

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