Witman Lab Publications

UMMS Affiliation

Department of Cell Biology

Publication Date


Document Type



Animals; Biological Transport; Cellular Structures; Chlamydomonas reinhardtii; Cilia; Flagella; Gene Deletion; Membranes; Microscopy, Immunoelectron; Microtubule-Associated Proteins; Microtubules; Mutation


Cell Biology


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.

DOI of Published Version



J Cell Biol. 2010 Sep 6;190(5):927-40. Link to article on publisher's site

Journal/Book/Conference Title

The Journal of cell biology

Related Resources

Link to Article in PubMed

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