An essential quality control mechanism at the eukaryotic basal body prior to intraflagellar transport.

Stephan, Angela and Vaughan, Sue and Shaw, Michael K. and Gull, Keith and McKean, Paul G. (2007) An essential quality control mechanism at the eukaryotic basal body prior to intraflagellar transport. Traffic, 8 (10). pp. 1323-1330. ISSN 1398-9219

Full text not available from this repository.


Constructing a eukaryotic cilium/flagellum is a demanding task requiring the transport of proteins from their cytoplasmic synthesis site into a spatially and environmentally distinct cellular compartment. The clear potential hazard is that import of aberrant proteins could seriously disable cilia/flagella assembly or turnover processes. Here, we reveal that tubulin protein destined for incorporation into axonemal microtubules interacts with a tubulin cofactor C (TBCC) domain-containing protein that is specifically located at the mature basal body transitional fibres. RNA interference-mediated ablation of this protein results in axonemal microtubule defects but no effect on other microtubule populations within the cell. Bioinformatics analysis indicates that this protein belongs to a clade of flagellum-specific TBCC-like proteins that includes the human protein, XRP2, mutations which lead to certain forms of the hereditary eye disease retinitis pigmentosa. Taken with other observations regarding the role of transitional fibres in cilium/flagellum assembly, we suggest that a localized protein processing capacity embedded at transitional fibres ensures the ‘quality’ of tubulin imported into the cilium/flagellum, and further, that loss of a ciliary/flagellar quality control capability may underpin a number of human genetic disorders.

Item Type: Journal Article
Journal or Publication Title: Traffic
Uncontrolled Keywords: /dk/atira/pure/researchoutput/libraryofcongress/qh301
Departments: Faculty of Health and Medicine > Biomedical & Life Sciences
ID Code: 9241
Deposited By: Dr Paul G McKean
Deposited On: 02 Jun 2008 12:48
Refereed?: Yes
Published?: Published
Last Modified: 07 Jan 2020 02:58

Actions (login required)

View Item View Item