Chlamydomonas IFT88 and Its Mouse Homologue, Polycystic Kidney Disease Gene Tg737, Are Required for Assembly of Cilia and Flagella

doi:10.1083/jcb.151.3.709

Published online 30 October 2000. doi:10.1083/jcb.151.3.709

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© The Rockefeller University Press, 0021-9525/2000//709 $5.00
The Journal of Cell Biology, Volume 151, Number 3, , 2000 709-718

Original Article

Chlamydomonas IFT88 and Its Mouse Homologue, Polycystic Kidney Disease Gene Tg737, Are Required for Assembly of Cilia and Flagella

Gregory J. Pazour^a, Bethany L. Dickert^a, Yvonne Vucica^b, E. Scott Seeley^b, Joel L. Rosenbaum^b, George B. Witman^a, and Douglas G. Cole^c

^a Department of Cell Biology, University of Massachusetts Medical School, Worcester, Massachusetts 01655
^b Department of Molecular, Cellular, and Developmental Biology, Yale University, New Haven, Connecticut 06520
^c Department of Microbiology, Molecular Biology, and Biochemistry, University of Idaho, Moscow, Idaho 83844
Department of Microbiology, Molecular Biology, and Biochemistry, Life Science South 142, University of Idaho, Moscow, ID 83844-3052.208-885-6518208-885-4071

Intraflagellar transport (IFT) is a rapid movement of multi-subunitprotein particles along flagellar microtubules and is requiredfor assembly and maintenance of eukaryotic flagella. We clonedand sequenced a Chlamydomonas cDNA encoding the IFT88 subunitof the IFT particle and identified a Chlamydomonas insertionalmutant that is missing this gene. The phenotype of this mutantis normal except for the complete absence of flagella. IFT88is homologous to mouse and human genes called Tg737. Mice withdefects in Tg737 die shortly after birth from polycystic kidneydisease. We show that the primary cilia in the kidney of Tg737mutant mice are shorter than normal. This indicates that IFTis important for primary cilia assembly in mammals. It is likelythat primary cilia have an important function in the kidneyand that defects in their assembly can lead to polycystic kidneydisease.

Key Words: orpk • intraflagellar transport • primary cilia • kinesin-II • cytoplasmic dynein

Mr. Seeley's current address is Dartmouth Medical School, Departmentof Biochemistry, Hanover, NH 03755.

Ms. Vucica's current address is Cell Biology Group, School ofPlant Science, The University of Tasmania, Hobart, Tasmania7001, Australia.

Abbreviations used in this paper: IFT, intraflagellar transport;TPR, tetratricopeptide repeats.

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