Ontology highlight
ABSTRACT:
SUBMITTER: Liu P
PROVIDER: S-EPMC5798339 | biostudies-other | 2018 Jan
REPOSITORIES: biostudies-other
Proceedings of the National Academy of Sciences of the United States of America 20180116 5
Bardet-Biedl syndrome (BBS) is a ciliopathy resulting from defects in the BBSome, a conserved protein complex. BBSome mutations affect ciliary membrane composition, impairing cilia-based signaling. The mechanism by which the BBSome regulates ciliary membrane content remains unknown. <i>Chlamydomonas bbs</i> mutants lack phototaxis and accumulate phospholipase D (PLD) in the ciliary membrane. Single particle imaging revealed that PLD comigrates with BBS4 by intraflagellar transport (IFT) while IF ...[more]