Ontology highlight
ABSTRACT:
SUBMITTER: Liu YP
PROVIDER: S-EPMC4001542 | biostudies-literature | 2014 May
REPOSITORIES: biostudies-literature
Liu Yangfan P YP Tsai I-Chun IC Morleo Manuela M Oh Edwin C EC Leitch Carmen C CC Massa Filomena F Lee Byung-Hoon BH Parker David S DS Finley Daniel D Zaghloul Norann A NA Franco Brunella B Katsanis Nicholas N
The Journal of clinical investigation 20140401 5
Cilia are critical mediators of paracrine signaling; however, it is unknown whether proteins that contribute to ciliopathies converge on multiple paracrine pathways through a common mechanism. Here, we show that loss of cilopathy-associated proteins Bardet-Biedl syndrome 4 (BBS4) or oral-facial-digital syndrome 1 (OFD1) results in the accumulation of signaling mediators normally targeted for proteasomal degradation. In WT cells, several BBS proteins and OFD1 interacted with proteasomal subunits, ...[more]