The C-elegans homolog of the murine cystic kidney disease gene Tg737 functions in a ciliogenic pathway and is disrupted in osm-5 mutant worms

Citation
Cj. Haycraft et al., The C-elegans homolog of the murine cystic kidney disease gene Tg737 functions in a ciliogenic pathway and is disrupted in osm-5 mutant worms, DEVELOPMENT, 128(9), 2001, pp. 1493-1505
Citations number
54
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Cell & Developmental Biology
Journal title
DEVELOPMENT
ISSN journal
0950-1991 → ACNP
Volume
128
Issue
9
Year of publication
2001
Pages
1493 - 1505
Database
ISI
SICI code
0950-1991(200105)128:9<1493:TCHOTM>2.0.ZU;2-Y
Abstract
Cilia and flagella are important organelles involved in diverse functions s uch as fluid and cell movement, sensory perception and embryonic patterning . They are devoid of protein synthesis, thus their formation and maintenanc e requires the movement of protein complexes from the cytoplasm into the ci lium and flagellum axoneme by intraflagellar transport (IFT), a conserved p rocess common to all ciliated or flagellated eukaryotic cells. We report th at mutations in the Caenorhabditis elegans gene Y41g9a.1 are responsible fo r the ciliary defects in osm-5 mutant worms. This was confirmed by transgen ic rescue of osm-5(p813) mutants using the wild-type Y41g9a.1 gene, osm-5 e ncodes a tetratricopeptide repeat (TPR)-containing protein that is the homo log of murine polaris (Tg737), a protein associated with cystic kidney dise ase and left-right axis patterning defects in the mouse, osm-5 is expressed in ciliated sensory neurons in C, elegans and its expression is regulated by DAF-19, an RFX-type transcription factor that governs the expression of other genes involved in cilia formation in the worm. Similar to murine pola ris, the OSM-5 protein was found to concentrate at the cilium base and with in the cilium axoneme as shown by an OSM-5::GFP translational fusion and im munofluorescence. Furthermore, time-lapse imaging of OSM-5::GFP fusion prot ein shows fluorescent particle migration within the cilia, Overall, the dat a support a crucial role for osm-5 in a conserved ciliogenic pathway, most likely as a component of the IFT process.