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10.1073/pnas.1713226115

http://scihub22266oqcxt.onion/10.1073/pnas.1713226115
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suck abstract from ncbi


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pmid29339469
      Proc+Natl+Acad+Sci+U+S+A 2018 ; 115 (5 ): E934-E943
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  • The Bardet-Biedl syndrome protein complex is an adapter expanding the cargo range of intraflagellar transport trains for ciliary export #MMPMID29339469
  • Liu P ; Lechtreck KF
  • Proc Natl Acad Sci U S A 2018[Jan]; 115 (5 ): E934-E943 PMID29339469 show ga
  • 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. Chlamydomonas bbs 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 IFT of PLD is abolished in bbs mutants. BBSome deficiency did not alter the rate of PLD entry into cilia. Membrane association and the N-terminal 58 residues of PLD are sufficient and necessary for BBSome-dependent transport and ciliary export. The replacement of PLD's ciliary export sequence (CES) caused PLD to accumulate in cilia of cells with intact BBSomes and IFT. The buildup of PLD inside cilia impaired phototaxis, revealing that PLD is a negative regulator of phototactic behavior. We conclude that the BBSome is a cargo adapter ensuring ciliary export of PLD on IFT trains to regulate phototaxis.
  • |*Mutation [MESH]
  • |Bardet-Biedl Syndrome/*genetics/*metabolism [MESH]
  • |Biological Transport [MESH]
  • |Cell Membrane/metabolism [MESH]
  • |Chlamydomonas reinhardtii/genetics/physiology [MESH]
  • |Cilia/*metabolism [MESH]
  • |Flagella/metabolism [MESH]
  • |Fluorescence Recovery After Photobleaching [MESH]
  • |Humans [MESH]
  • |Phospholipase D/metabolism [MESH]
  • |Photochemical Processes [MESH]
  • |Phototaxis [MESH]
  • |Protein Domains [MESH]
  • |Protein Transport/*genetics [MESH]
  • |Proteins/metabolism [MESH]


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