Intraflagellar transport and cilia-dependent diseases.
Intraflagellar transport involves the movement of large protein particles along ciliary microtubules and is required for the assembly and maintenance of eukaryotic cilia and flagella. Intraflagellar-transport defects in the mouse cause a range of diseases including polycystic kidney disease, retinal degeneration and the laterality abnormality situs inversus, highlighting the important role that motile, sensory and primary cilia play in vertebrates.
Pazour
Gregory J
GJ
Dept Cell Biology, University of Massachusetts Medical School, Worcester, MA 01655, USA. gregory.pazour@umassmed.edu
Rosenbaum
Joel L
JL
D000818:Animals; D001692:Biological Transport; D002923:Cilia / Q000378:metabolism* / Q000648:ultrastructure; D005407:Flagella / Q000502:physiology* / Q000648:ultrastructure; D006801:Humans; D007690:Polycystic Kidney Diseases / Q000378:metabolism* / Q000648:ultrastructure; D012162:Retinal Degeneration / Q000378:metabolism*; D012857:Situs Inversus / Q000378:metabolism*