LB Pedersen

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We reported earlier that CSPP-L, the larger isoform of the Centrosome/Spindle Pole associated Protein 1, is a ciliary protein required for primary cilia formation in hTERT-RPE1 cells. CSPP-L was found to localize to the basal body and the proximal and distal end of the primary cilium, and to be essential for recruitment of its interaction partner NPHP8(More)
Caenorhabditis elegans kinesin-3 KLP-6 localises to cilia and regulates ciliary localization of polycystins [1]. Here we investigated if kinesin-3 motors also have cilia-related functions in vertebrates. We used transcriptomics to identify kinesins that are upregulated during serum deprivation and ciliogenesis in mouse fibroblasts and identified Kif13b(More)
Objective The receptor tyrosine kinase (RTK) Platelet-Derived Growth Factor Receptor a (PDGFRa) localizes to the primary cilium, and expression of the constitutive active PDGFRa D842V mutant has been linked to gastrointest-inal tumor formation. We set out to investigate whether expression of D842V causes loss of primary cilia in cultured cells. Methods(More)
Objective Centrosome/Spindle Pole associated Protein 1 (CSPP1, JBTS21) mutations cause Joubert syndrome (JBTS) and JBTS-related ciliopathies. The large protein isoform CSPP-L is a ciliary protein required for ciliogenesis and stabilization of the ciliopathy protein RPGRIP1L (NPHP8/JBTS7/MKS5/FTM) at the ciliary transition zone (TZ). However, RPGRIP1L is(More)
We previously showed that canonical TGFb signaling is regulated in part by the primary cilium, and that ciliary TGFb signaling is upregulated in stem cells differentiating into cardiomyocytes [1]. Ciliary signaling was shown to be associated with clathrin-dependent endocytosis at the ciliary pocket for activation of SMAD2/3 transcription factors that(More)
In Caenorhabditis elegans the kinesin-3 Klp6 is required for localization of polycystin-2 to sensory cilia (Peden and Barr, 2005) and regulation of IFT (Morsci and Barr, 2011). A kinesin-3-encoding gene from Drosophila contains a consensus binding site for cilia-specific RFX-type transcription factors, suggesting a potential ciliary function for this(More)
TGFb signaling extensively cross-talks with Hh, Wnt and RTK signaling to control cell proliferation, migration and differentiation, and when aberrantly regulated leads to developmental defects and cancer. TGFb signaling is activated through the internalization of TGFb receptors via clathrin-dependent endocytosis (CDE), at which the receptor activates Smad(More)
The mother centriole forms the basis of the primary cilium. As the cilium assembles, the mother centriole matures and differentiates into the basal body, and a number of fibrous structures are formed that add to the complexity , including the distal and subdistal appendages. A number of the proteins corresponding to these structures are identified already,(More)
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