D103 Lecture 18.1- primary cilia

D103 Lecture 18.1- primary cilia - Bio D103 Cell Biology...

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Bio D103: Cell Biology Instructor: Dr. Scott Atwood Fall 2016
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After reading the paired chapters , you should be able to: Understand the structure and function of cilia Describe the role of cilia in development and disease After reviewing this lecture , you should be able to: Describe the BBSome and its functions Understand the structure of primary cilia and how they move Describe how intraflagellar transport works and how it relates to Hedgehog signaling Lecture 18: Learning objectives
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Meet Robbie, he has Bardet-Biedl Syndrome (BBS) http://www.ntnews.com.au/news/northern-territory/darwin-baby-robbie-celebrates-first-birthday-against-all-odds/story-fnk0b1zt-1226935990509
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Bardet-Biedl Syndrome (BBS) diagnosis typically, patients eventually go blind, deaf, and have lasting kidney defects and learning disabilities
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BBSome (a complex of BBS proteins) Ciliopathies : MKS : Meckel-Gruber syndrome, MKKS : McKusick-Kaufman syndrome, JBTS : Joubert syndrome, NPHP : nephronophthisis, SLS : Senior- Loken syndrome, RP : retinitis pigmentosa, LCA : Leber congenital amaurosis
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BBS a new coat for vesicular traffic to the primary cilium?
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Wyman Manderly is interested in the BBSome complex because of his family history. How would he know whether the BBSome is likely a coat complex?
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