<p><b>A</b>) RPE-1 cells expressing GFP-BBS4 were depleted of AZI1, and the number of cells with ciliary GFP (BBS4) was counted. BBS4 is stained with GFP (green), Acetylated α-tubulin was used to detect cilia (red). <b>B</b>) Graph showing significant increases in ciliary localization of BBS4 upon AZI1 knockdown. <b>C</b>) Depletion of AZI1 in BBS3 and BBS5 depleted cells restores ciliary BBSome localization. RPE-1 cells were transfected with siRNA as indicated, and BBS9 (red) localization was analyzed. Cilia (green) in the insets of figures <b>A</b> and <b>C</b> are slightly shifted to show ciliary localization of BBS proteins. <b>D</b>) Cilia containing BBS9 at different conditions were counted and presented graphically. All data are pres...
SummaryPrimary cilium dysfunction underlies the pathogenesis of Bardet-Biedl syndrome (BBS), a genet...
Primary cilia are sensory organelles vital for developmental and physiological processes. Their dysf...
Primary cilia are sensory organelles vital for developmental and physiological processes. Their dysf...
<p><b>A</b>) RPE-1 cells were transfected with siRNA against <i>BBS4</i>, <i>PCM1</i>, and <i>AZI1</...
<p><b>A</b>) AZI1 and PCM1 co-localize at the centrosome. IMCD3 cells were transfected with HA-AZI1 ...
<p>Mouse ShhLIGHT-II fibroblast cells were transfected with siRNA (a non-targeting control siRNA (Ct...
Bardet-Biedl syndrome (BBS) is a well-known ciliopathy with mutations reported in 18 different genes...
<p>(A–R) Control RPE1 cells (A, D, G, J, M, and P) or the <i>BBS1</i>-KO cell lines #B1-1-23 (B, E, ...
<div><p>Defects in cilium and centrosome function result in a spectrum of clinically-related disorde...
<p><b>A</b>) A silver stained gel from GFP IP sample of testis lysate. Lane 1 shows the wild-type (W...
<p>(A–F) hTERT-RPE1 cells, (A) stained with anti-polyglutamylated tubulin (PGT), marking the cilium ...
<p>(A) In mammalian somatic cells, Azi1/Cep131 is a highly conserved and ubiquitously expressed coil...
<p><b>A</b>) 1. Partial BBSome complex (without BBS4) arrives near the centrosome, where BBS4 is par...
<p>(A and B) Knockdown of Dzip1 impairs ciliary localization of Rab8. RNAi control (Con) and Dzip1 s...
<p>(A–G) Immunofluorescent staining of hTERT-RPE1 cells transfected with empty vector (EV), sh<i>BBS...
SummaryPrimary cilium dysfunction underlies the pathogenesis of Bardet-Biedl syndrome (BBS), a genet...
Primary cilia are sensory organelles vital for developmental and physiological processes. Their dysf...
Primary cilia are sensory organelles vital for developmental and physiological processes. Their dysf...
<p><b>A</b>) RPE-1 cells were transfected with siRNA against <i>BBS4</i>, <i>PCM1</i>, and <i>AZI1</...
<p><b>A</b>) AZI1 and PCM1 co-localize at the centrosome. IMCD3 cells were transfected with HA-AZI1 ...
<p>Mouse ShhLIGHT-II fibroblast cells were transfected with siRNA (a non-targeting control siRNA (Ct...
Bardet-Biedl syndrome (BBS) is a well-known ciliopathy with mutations reported in 18 different genes...
<p>(A–R) Control RPE1 cells (A, D, G, J, M, and P) or the <i>BBS1</i>-KO cell lines #B1-1-23 (B, E, ...
<div><p>Defects in cilium and centrosome function result in a spectrum of clinically-related disorde...
<p><b>A</b>) A silver stained gel from GFP IP sample of testis lysate. Lane 1 shows the wild-type (W...
<p>(A–F) hTERT-RPE1 cells, (A) stained with anti-polyglutamylated tubulin (PGT), marking the cilium ...
<p>(A) In mammalian somatic cells, Azi1/Cep131 is a highly conserved and ubiquitously expressed coil...
<p><b>A</b>) 1. Partial BBSome complex (without BBS4) arrives near the centrosome, where BBS4 is par...
<p>(A and B) Knockdown of Dzip1 impairs ciliary localization of Rab8. RNAi control (Con) and Dzip1 s...
<p>(A–G) Immunofluorescent staining of hTERT-RPE1 cells transfected with empty vector (EV), sh<i>BBS...
SummaryPrimary cilium dysfunction underlies the pathogenesis of Bardet-Biedl syndrome (BBS), a genet...
Primary cilia are sensory organelles vital for developmental and physiological processes. Their dysf...
Primary cilia are sensory organelles vital for developmental and physiological processes. Their dysf...