CEP290 tethers flagellar transition zone microtubules to the membrane and regulates flagellar protein content
Authors
Craige, BranchTsao, Che-Chia
Diener, Dennis R.
Hou, Yuqing
Lechtreck, Karl-Ferdinand
Rosenbaum, Joel L.
Witman, George B.
UMass Chan Affiliations
Department of Cell BiologyDocument Type
Journal ArticlePublication Date
2010-09-08Keywords
AnimalsBiological Transport
Cellular Structures
Chlamydomonas reinhardtii
Cilia
Flagella
Gene Deletion
Membranes
Microscopy, Immunoelectron
Microtubule-Associated Proteins
Microtubules
Mutation
Cell Biology
Metadata
Show full item recordAbstract
Mutations in human CEP290 cause cilia-related disorders that range in severity from isolated blindness to perinatal lethality. Here, we describe a Chlamydomonas reinhardtii mutant in which most of the CEP290 gene is deleted. Immunoelectron microscopy indicated that CEP290 is located in the flagellar transition zone in close association with the prominent microtubule-membrane links there. Ultrastructural analysis revealed defects in these microtubule-membrane connectors, resulting in loss of attachment of the flagellar membrane to the transition zone microtubules. Biochemical analysis of isolated flagella revealed that the mutant flagella have abnormal protein content, including abnormal levels of intraflagellar transport proteins and proteins associated with ciliopathies. Experiments with dikaryons showed that CEP290 at the transition zone is dynamic and undergoes rapid turnover. The results indicate that CEP290 is required to form microtubule-membrane linkers that tether the flagellar membrane to the transition zone microtubules, and is essential for controlling flagellar protein composition.Source
J Cell Biol. 2010 Sep 6;190(5):927-40. Link to article on publisher's siteDOI
10.1083/jcb.201006105Permanent Link to this Item
http://hdl.handle.net/20.500.14038/51085Related Resources
Link to Article in PubMedae974a485f413a2113503eed53cd6c53
10.1083/jcb.201006105