2007RePub (Erasmus University Rotterdam)Open access

Ciliary Length Control

Joost R. Broekhuis

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Abstract

Primary cilia are microtubule-based organelles that can be found on the surface of most\neukaryotic cells. Primary cilia detect external cues and play an important role in subsequent\nsignal transduction pathways. In the kidneys primary cilia act as a flow sensor. However,\nboth abnormally short and excessively long cilia fail to correctly act as a flow sensor, which\nresults in the formation of cysts inside the kidney and eventually kidney failure. Primary cilia\nare assembled and maintained by a process known as intraflagellar transport. This process\ninvolves the bidirectional transport of multimeric protein complexes along the microtubule\naxis by motor proteins. It is likely that lengthening and shrinkage of cilia is modulated by the\nintraflagellar transport machinery. This thesis contains studies aimed to gain more insight in\nhow intraflagellar transport and cilia length are regulated. To study intraflagellar transport we\nuse cultured mammalian cells as well as the nematode Caenorhabditis elegans.

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Primary cilia are microtubule-based organelles that can be found on the surface of most\neukaryotic cells. Primary cilia detect external cues and play an important role in subsequent\nsignal transduction pathways. In the kidneys primary cilia act as a flow sensor. However,\nboth abnormally short and excessively long cilia fail to correctly act as a flow sensor, which\nresults in the formation of cysts inside the kidney and eventually kidney failure. Primary cilia\nare assembled and maintained by a process known as intraflagellar transport. This process\ninvolves the bidirectional transport of multimeric protein complexes along the microtubule\naxis by motor proteins. It is likely that lengthening and shrinkage of cilia is modulated by the\nintraflagellar transport machinery. This thesis contains studies aimed to gain more insight in\nhow intraflagellar transport and cilia length are regulated. To study intraflagellar transport we\nuse cultured mammalian cells as well as the nematode Caenorhabditis elegans.

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Available abstract

Primary cilia are microtubule-based organelles that can be found on the surface of most\neukaryotic cells. Primary cilia detect external cues and play an important role in subsequent\nsignal transduction pathways. In the kidneys primary cilia act as a flow sensor. However,\nboth abnormally short and excessively long cilia fail to correctly act as a flow sensor, which\nresults in the formation of cysts inside the kidney and eventually kidney failure. Primary cilia\nare assembled and maintained by a process known as intraflagellar transport. This process\ninvolves the bidirectional transport of multimeric protein complexes along the microtubule\naxis by motor proteins. It is likely that lengthening and shrinkage of cilia is modulated by the\nintraflagellar transport machinery. This thesis contains studies aimed to gain more insight in\nhow intraflagellar transport and cilia length are regulated. To study intraflagellar transport we\nuse cultured mammalian cells as well as the nematode Caenorhabditis elegans.

Key concepts: Intraflagellar transport, Cilium, Cell biology, Microtubule, Dynein, Biology, Caenorhabditis elegans, Kinesin

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