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Academic Background

BS: 1986, South Dakota State University

PhD: 1991, University of Minnesota

Post Doctoral Studies: Worcester Foundation for Biomedical Research

Function of the Mammalian Primary Cilium and Mechanisms of Eukaryotic Ciliary Assembly

The Pazour lab is interested in understanding the mechanism for assembly of eukaryotic cilia and the function that these organelles play in vertebrate health and development.  Across the eukaryotic kingdom, cilia function to produce force for cell motility and serve as cellular antennae allowing cells to sense the environment.  In vertebrates, cilia play critical roles in development by establishing the left-right pattern of organ development and in organizing hedgehog and other signaling pathways.  Ciliary dysfunction leads to a spectrum of structural birth defects affecting every organ of the body. In addition, our senses of sight and smell utilize ciliary localized receptors and thus ciliary defects can lead to anosmia and blindness.  Post development, cilia play critical roles in tissue homeostasis and ciliary dysfunction causes a number of serious adult onset diseases including polycystic kidney disease, obesity and cancers such as medulloblastoma and basal cell carcinoma.

In the kidney, the epithelial cells of uriniferous tubule have prominent primary cilia extending from their apical surface into the lumen.  The precise function of the cilia is unknown but they are thought to monitor the tubule diameter. If the cilia are defective, the tubule epithelial cell over proliferate and transform the normally narrow-lumen tubule into a large cyst.  For more information see "Chlamydomonas IFT88 and its mouse homologue, polycystic kidney disease gene Tg737, are required for assembly of cilia and flagella"

 Tg737 Scanning EM

 IFT20 Deletion Histology

In the heart, cilia (green in the image below) play critical roles in left-right patterning and hedgehog signaling.  Ciliary dysfunction causes severe structural cardiac malformations and is thought to be a major cause of structural heart disease in the human population.  For more information see "Global genetic analysis in mice unveils central role for cilia in congenital heart disease"

 

 

One or more keywords matched the following items that are connected to Pazour, Gregory
Item TypeName
Academic Article The vertebrate primary cilium is a sensory organelle.
Academic Article Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis: summary statement of a first National Institutes of Health/Office of Rare Diseases conference.
Academic Article The cytoplasmic tail of fibrocystin contains a ciliary targeting sequence.
Academic Article IFT20 links kinesin II with a mammalian intraflagellar transport complex that is conserved in motile flagella and sensory cilia.
Academic Article Localization of transient receptor potential ion channels in primary and motile cilia of the female murine reproductive organs.
Academic Article PDGFRalphaalpha signaling is regulated through the primary cilium in fibroblasts.
Academic Article The primary cilium coordinates early cardiogenesis and hedgehog signaling in cardiomyocyte differentiation.
Academic Article Intraflagellar transport is required for polarized recycling of the TCR/CD3 complex to the immune synapse.
Academic Article IFT25 links the signal-dependent movement of Hedgehog components to intraflagellar transport.
Academic Article Analysis of ciliary membrane protein dynamics using SNAP technology.
Concept Receptors, Antigen, T-Cell
Concept Receptor, Platelet-Derived Growth Factor alpha
Concept Receptors, Cell Surface
Concept Platelet-Derived Growth Factor
Concept Receptors, G-Protein-Coupled
Concept alpha-Galactosidase
Concept Receptor Tyrosine Kinase-like Orphan Receptors
Concept Receptors, Immunologic
Academic Article Specific recycling receptors are targeted to the immune synapse by the intraflagellar transport system.
Academic Article Arf4 is required for Mammalian development but dispensable for ciliary assembly.
Academic Article IFT27 links the BBSome to IFT for maintenance of the ciliary signaling compartment.
Academic Article Intraflagellar transport 27 is essential for hedgehog signaling but dispensable for ciliogenesis during hair follicle morphogenesis.
Academic Article The small GTPase Rab8 interacts with VAMP-3 to regulate the delivery of recycling T-cell receptors to the immune synapse.
Academic Article IFT20 controls LAT recruitment to the immune synapse and T-cell activation in vivo.
Academic Article Ror2 signaling regulates Golgi structure and transport through IFT20 for tumor invasiveness.
Academic Article BLOC-1 is required for selective membrane protein trafficking from endosomes to primary cilia.
Academic Article Super-resolution microscopy reveals that disruption of ciliary transition-zone architecture causes Joubert?syndrome.
Academic Article Intraflagellar transport is deeply integrated in hedgehog signaling.
Academic Article Cilia in cystic kidney and other diseases.
Academic Article Ubiquitin links smoothened to intraflagellar transport to regulate Hedgehog signaling.
Academic Article E3 ubiquitin ligase Wwp1 regulates ciliary dynamics of the Hedgehog receptor Smoothened.
Concept Patched Receptors
Concept Patched-1 Receptor
Concept Smoothened Receptor
Academic Article Biallelic pathogenic variants in roundabout guidance receptor 1 associate with syndromic congenital anomalies of the kidney and urinary tract.
Academic Article Arih2 regulates Hedgehog signaling through smoothened ubiquitylation and ER-associated degradation.
Search Criteria
  • Receptor
  • Platelet Derived Growth Factor
  • alpha