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OUR RESEARCH

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Proximity-Dependent Biotin Identification (BioID)

Proximity-dependent biotin identification (BioID) is a novel pipeline developed by Dr. Brian Raught at Princess Margaret Cancer Centre, Toronto.  This method relies on a mutant form of the biotin

ligase BirA that promiscuously biotinylates proximal proteins. To identify protein interactors, we will generate cell lines with inducible BirA fusion proteins expressed from a defined genomic locus.

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The Fault In Our CFTRs: Interactome Modelling of CFTR

Cystic fibrosis (CF), one of the most common inherited childhood diseases, is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that encodes a chloride ion channel.  A general hypothesis in my approach to combat CF is that the success of therapy will be related to the extent to which it restores missing or aberrant CFTR protein interactions.

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The Long-Lost Organelle: The Primary Cilium

Primary cilia are cellular ‘antennae’ that mediate critical cell signaling pathways (e.g. Hedgehog, Wnt and PDGF). Ciliary membranes must generate optimal concentrations of receptors to correctly interpret diverse extracellular cues. Ciliary defects lead to a panoply of human diseases, termed ciliopathies, and the loss of this organelle may be a key event during oncogenic transformation1. By understanding how the ciliary membrane and underlying structure are assembled, we will provide fundamental insights on cellular growth and signaling.

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Polycystic Kidney Disease: Identifying the Players

Of the many clinical features of ciliopathies, a major hallmark is renal cystic disease. Polycystic Kidney Disease (PKD) results from mutations in Polycystic Kidney Disease 1 (PKD1), PKD2, or Polycystic Kidney and Hepatic Disease 1 (PKHD1) genes. ~85% of autosomal dominant polycystic kidney disease (ADPKD) is secondary to mutations in PKD1. Our hypothesis is that ciliary dysfunction may present a mechanism for ADPKD. In support of this, multiple signaling pathways are disrupted in ADPKD and accumulating evidence suggests that a number of these are mediated by primary cilia.

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