Research Topics
Genomes and Genes | G J PazourSummaryAffiliation: University of Massachusetts Medical School Country: USA Publications
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Publications
Disruption of IFT complex A causes cystic kidneys without mitotic spindle misorientationJulie A Jonassen
Department of Microbiology and Physiological Systems, University of Massachusetts Medical School, Worcester, MA 01605, USA
J Am Soc Nephrol 23:641-51. 2012..Taken together, these studies indicate that loss of Ift140 causes pronounced renal cystic disease and suggest that abnormalities in several different pathways may influence cyst progression...
Forward and reverse genetic analysis of microtubule motors in ChlamydomonasG J Pazour
Department of Cell Biology, University of Massachusetts Medical School, 55 Lake Avenue North, Worcester, Massachusetts 01655, USA
Methods 22:285-98. 2000..We describe methods to generate and characterize insertional mutants, using mutations that affect the outer dynein arm as examples...- Intraflagellar transport and cilia-dependent renal disease: the ciliary hypothesis of polycystic kidney diseaseGregory J Pazour
Program in Molecular Medicine, University of Massachusetts Medical School, Biotech II, Suite 213, 373 Plantation Street, Worcester 01605, USA
J Am Soc Nephrol 15:2528-36. 2004.... - Intraflagellar transport and cilia-dependent diseasesGregory J Pazour
Dept Cell Biology, University of Massachusetts Medical School, Worcester, MA 01655, USA
Trends Cell Biol 12:551-5. 2002.... - The vertebrate primary cilium is a sensory organelleGregory J Pazour
Program in Molecular Medicine, University of Massachusetts Medical School, 373 Plantation Street, Worcester, MA 01605, USA
Curr Opin Cell Biol 15:105-10. 2003..Primary cilia on other cell types specifically display different receptors, including those for somatostatin and serotonin... - Comparative genomics: prediction of the ciliary and basal body proteomeGregory J Pazour
Program in Molecular Medicine, University of Massachusetts Medical School, Biotech II, Suite 213, 373 Plantation St, Worcester, Massachusetts 01605, USA
Curr Biol 14:R575-7. 2004..Two recent studies used comparative genomics to predict the ciliary and basal body proteomes, providing datasets that are rich sources of human disease gene candidates... 
Chlamydomonas IFT88 and its mouse homologue, polycystic kidney disease gene tg737, are required for assembly of cilia and flagellaG J Pazour
Department of Cell Biology, University of Massachusetts Medical School, Worcester, Massachusetts 01655, USA
J Cell Biol 151:709-18. 2000..This indicates that IFT is important for primary cilia assembly in mammals. It is likely that primary cilia have an important function in the kidney and that defects in their assembly can lead to polycystic kidney disease...- The Chlamydomonas reinhardtii BBSome is an IFT cargo required for export of specific signaling proteins from flagellaKarl Ferdinand Lechtreck
Department of Cell Biology, University of Massachusetts Medical School, Worcester, 01655, USA
J Cell Biol 187:1117-32. 2009..We conclude that the BBSome is carried by IFT but is an adapter rather than an integral component of the IFT machinery. C. reinhardtii BBS4 may be required for the export of signaling proteins from the flagellum via IFT... - Proteomic analysis of a eukaryotic ciliumGregory J Pazour
Program in Molecular Medicine, University of Massachusetts Medical School, Worcester, MA 01605, USA
J Cell Biol 170:103-13. 2005..The flagellum also contains many proteins that are conserved in humans but have not been previously characterized in any organism. The results indicate that flagella are far more complex than previously estimated... - A dynein light intermediate chain, D1bLIC, is required for retrograde intraflagellar transportYuqing Hou
Department of Cell Biology, University of Massachusetts Medical School, Worcester, MA 01655, USA
Mol Biol Cell 15:4382-94. 2004..The constructs rescued the mutant cells to a wild-type phenotype, indicating that the function of D1bLIC in IFT is independent of its P-loop... - IFT20 links kinesin II with a mammalian intraflagellar transport complex that is conserved in motile flagella and sensory ciliaSheila A Baker
Department of Cell Biology, Neurobiology, and Anatomy, Medical College of Wisconsin, Milwaukee, Wisconsin 53226, USA
J Biol Chem 278:34211-8. 2003..Our data indicate that all four mammalian IFT proteins are part of a highly conserved complex in multiple ciliated cell types. Furthermore, IFT20 appears to bridge kinesin II with the IFT complex... - Photoreceptor intersegmental transport and retinal degeneration: a conserved pathway common to motile and sensory ciliaJoseph C Besharse
Department of Cell Biology, Neurobiology and Anatomy, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53226, USA
Adv Exp Med Biol 533:157-64. 2003 - The cytoplasmic tail of fibrocystin contains a ciliary targeting sequenceJohn A Follit
Program in Molecular Medicine, University of Massachusetts Medical School, Worcester, MA 01605, USA
J Cell Biol 188:21-8. 2010..This suggests that the CTS functions by binding regulatory proteins like Rab8 to control trafficking through the endomembrane system and on to the cilium... - Functional analysis of an individual IFT protein: IFT46 is required for transport of outer dynein arms into flagellaYuqing Hou
Department of Cell Biology, University of Massachusetts Medical School, Worcester, MA 01655, USA
J Cell Biol 176:653-65. 2007..Axonemal ultrastructure is restored, except that the outer arms are still missing, although outer arm subunits are present in the cytoplasm. Thus, IFT46 is specifically required for transporting outer arms into the flagellum... - DC3, the 21-kDa subunit of the outer dynein arm-docking complex (ODA-DC), is a novel EF-hand protein important for assembly of both the outer arm and the ODA-DCDiane M Casey
Department of Cell Biology, University of Massachusetts Medical School, Worcester, Massachusetts 01655, USA
Mol Biol Cell 14:3650-63. 2003..The results indicate that DC3 is important for both outer arm and ODA-DC assembly... - Pericentrin forms a complex with intraflagellar transport proteins and polycystin-2 and is required for primary cilia assemblyAgata Jurczyk
Department of Molecular Medicine, University of Massachusetts Medical School, 373 Plantation Street, Biotech II, Suite 210, Worcester, MA 01605, USA
J Cell Biol 166:637-43. 2004..We conclude that Pcnt, IFTs, and PC2 form a complex in vertebrate cells that is required for assembly of primary cilia and possibly motile cilia and flagella... 
Targeting proteins to the ciliary membraneGregory J Pazour
Program in Molecular Medicine, University of Massachusetts Medical School, Biotech II, Worcester, Massachusetts, USA
Curr Top Dev Biol 85:115-49. 2008..We will further discuss ongoing work to understand how the ciliary membrane is assembled and maintained, and discuss protein machinery that is thought to play a role in sorting or trafficking proteins to the ciliary membrane...- The Golgin GMAP210/TRIP11 anchors IFT20 to the Golgi complexJohn A Follit
Program in Molecular Medicine, University of Massachusetts Medical School, Worcester, Massachusetts, USA
PLoS Genet 4:e1000315. 2008..This work suggests that GMAP210 and IFT20 function together at the Golgi in the sorting or transport of proteins destined for the ciliary membrane... - Characterization of mouse IFT complex BJohn A Follit
Program in Molecular Medicine, University of Massachusetts Medical School, Worcester, Massachusetts, USA
Cell Motil Cytoskeleton 66:457-68. 2009..This suggests that IFT54s effect on IFT20 is a dominant negative phenotype caused by its overexpression. Cell Motil. Cytoskeleton 2009. (c) 2009 Wiley-Liss, Inc... - Scanning electron microscopy to examine cells and organsJovenal T SanAgustin
Program in Molecular Medicine, University of Massachusetts Medical School, Worcester, Massachusetts 01605, USA
Methods Cell Biol 91:81-7. 2009..The methods provided here can be used with little modification to examine other mammalian organs or used as a starting point to develop methods for use in other organisms... - The intraflagellar transport protein IFT20 is associated with the Golgi complex and is required for cilia assemblyJohn A Follit
Program in Molecular Medicine, Department of Physiology, University of Massachusetts Medical School, Worcester, MA 01605, USA
Mol Biol Cell 17:3781-92. 2006..This work suggests that IFT20 functions in the delivery of ciliary membrane proteins from the Golgi complex to the cilium... - The intraflagellar transport protein, IFT88, is essential for vertebrate photoreceptor assembly and maintenanceGregory J Pazour
Department of Cell Biology, University of Massachusetts Medical School, Worcester, MA 01655, USA
J Cell Biol 157:103-13. 2002..We further find that mice with a mutation in the IFT particle protein gene, Tg737/IFT88, have abnormal OS development and retinal degeneration. Thus, IFT is important for assembly and maintenance of the vertebrate OS... - Deletion of IFT20 in the mouse kidney causes misorientation of the mitotic spindle and cystic kidney diseaseJulie A Jonassen
Program in Molecular Medicine, University of Massachusetts Medical School, Worcester, MA 01605, USA
J Cell Biol 183:377-84. 2008..At later stages, cells lacking cilia have increased canonical Wnt signaling and increased rates of proliferation. Thus, IFT20 functions to couple extracellular events to cell proliferation and differentiation... - Orpk mouse model of polycystic kidney disease reveals essential role of primary cilia in pancreatic tissue organizationDavid A Cano
Diabetes Center, Department of Medicine, University of California, San Francisco, CA 94143, USA
Development 131:3457-67. 2004..Thus, polaris and primary cilia function are required for the maturation and maintenance of proper tissue organization in the pancreas... - Meeting report: Tenth International Conference on the Cell and Molecular Biology of ChlamydomonasSaul Purton
Protist 153:325-36. 2002 - A genetic screen in zebrafish identifies cilia genes as a principal cause of cystic kidneyZhaoxia Sun
MIT, Center for Cancer Research and Biology Department, Building E17 Room 340, 77 Massachusetts Avenue, Cambridge, MA 02139, USA
Development 131:4085-93. 2004..Our results also suggest that lesions in genes involved in cilia formation and function are the predominant cause of cystic kidney disease, and that the genes identified here are excellent candidates for novel human PKD genes... - Polycystin-2 localizes to kidney cilia and the ciliary level is elevated in orpk mice with polycystic kidney diseaseGregory J Pazour
Curr Biol 12:R378-80. 2002 - The LC7 light chains of Chlamydomonas flagellar dyneins interact with components required for both motor assembly and regulationLinda M DiBella
Department of Biochemistry, University of Connecticut Health Center, Farmington, CT 06030-3305, USA
Mol Biol Cell 15:4633-46. 2004..These data suggest that LC7a stabilizes both the outer arms and inner arm I1 and that both LC7a and LC7b are involved in multiple intradynein interactions within both dyneins... - Radial spoke proteins of Chlamydomonas flagellaPinfen Yang
Department of Biological Sciences, Marquette University, Milwaukee, WI 53233, USA
J Cell Sci 119:1165-74. 2006..The human genes encoding these proteins are candidates for causing primary ciliary dyskinesia, a severe inherited disease involving missing or defective axonemal structures, including the radial spokes... - Function and dynamics of PKD2 in Chlamydomonas reinhardtii flagellaKaiyao Huang
Department of Molecular Cell and Developmental Biology, Yale University, New Haven, CT 06520, USA
J Cell Biol 179:501-14. 2007..These results suggest that the CrPKD2 cation channel is involved in coupling flagellar adhesion at the beginning of mating to the increase in flagellar calcium required for subsequent steps in mating... - The tiny eukaryote Ostreococcus provides genomic insights into the paradox of plankton speciationBrian Palenik
Scripps Institution of Oceanography, University of California at San Diego, La Jolla, CA 92093 0202, USA
Proc Natl Acad Sci U S A 104:7705-10. 2007..As reported here, selenoenzymes, novel fusion proteins, and loss of some major protein families including ones associated with chromatin are likely important adaptations for achieving a small cell size... - PDGFRalphaalpha signaling is regulated through the primary cilium in fibroblastsLinda Schneider
Department of Biochemistry, Institute for Molecular Biology and Physiology, University of Copenhagen, The August Krogh Building, Universitetsparken 13, DK 2100 Copenhagen Ø, Denmark
Curr Biol 15:1861-6. 2005..We propose that ciliary PDGFRalphaalpha signaling is linked to tissue homeostasis and to mitogenic signaling pathways... - Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis: summary statement of a first National Institutes of Health/Office of Rare Diseases conferenceMeral Gunay-Aygun
National Human Genome Research Institute, the Molecular Imaging Program, National Cancer Institute, The National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892-1851, USA
J Pediatr 149:159-64. 2006 - Nephrocystin specifically localizes to the transition zone of renal and respiratory cilia and photoreceptor connecting ciliaManfred Fliegauf
Department of Pediatrics and Adolescent Medicine, Mathildenstrasse 1, 79106 Freiburg, Germany
J Am Soc Nephrol 17:2424-33. 2006..In conclusion, nephrocystin deficiency or dysfunction at the transition zone of renal monocilia and the photoreceptor connecting cilium might explain renal failure and retinal degeneration that are observed in patients with NPHP1... - Localization of transient receptor potential ion channels in primary and motile cilia of the female murine reproductive organsStefan C Teilmann
Laboratory of Reproductive Biology, Rigshospitalet, Denmark
Mol Reprod Dev 71:444-52. 2005..In the oviduct both TRPV4 and polycystins could be important in relaying physiochemical changes in the oviduct upon ovulation...
Research Grants
- Intraflagellar Transport Proteins in MiceGregory J Pazour; Fiscal Year: 2011..This proposal seeks to understand the mechanism by which cilia are assembled and ultimately drive the identification of a treatment or cure for these diseases. ..
- Intraflagellar Transport Proteins in MiceGregory Pazour; Fiscal Year: 2009..The work proposed here will provide new information on how cilia are assembled and how defects in these organelles cause blindness and cystic kidney disease in humans. ..
- Intraflagellar Transport Proteins in MiceGregory Pazour; Fiscal Year: 2006..The work proposed here will provide new information on how cilia are assembled and how defects in these organelles cause blindness and cystic kidney disease in humans. ..
- Intraflagellar Transport Proteins in MiceGregory Pazour; Fiscal Year: 2005....
- Intraflagellar Transport Proteins in MiceGregory J Pazour; Fiscal Year: 2010..The work proposed here will provide new information on how cilia are assembled and how defects in these organelles cause blindness and cystic kidney disease in humans. ..