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dc.contributor.authorPazour, Gregory J.
dc.contributor.authorDickert, Bethany L.
dc.contributor.authorVucica, Yvonne
dc.contributor.authorSeeley, E. Scott
dc.contributor.authorRosenbaum, Joel L.
dc.contributor.authorWitman, George B.
dc.contributor.authorCole, Douglas G.
dc.date2022-08-11T08:10:05.000
dc.date.accessioned2022-08-23T16:54:51Z
dc.date.available2022-08-23T16:54:51Z
dc.date.issued2000-11-04
dc.date.submitted2008-08-15
dc.identifier.citationJ Cell Biol. 2000 Oct 30;151(3):709-18.
dc.identifier.issn0021-9525 (Print)
dc.identifier.pmid11062270
dc.identifier.urihttp://hdl.handle.net/20.500.14038/42594
dc.description.abstractIntraflagellar transport (IFT) is a rapid movement of multi-subunit protein particles along flagellar microtubules and is required for assembly and maintenance of eukaryotic flagella. We cloned and sequenced a Chlamydomonas cDNA encoding the IFT88 subunit of the IFT particle and identified a Chlamydomonas insertional mutant that is missing this gene. The phenotype of this mutant is normal except for the complete absence of flagella. IFT88 is homologous to mouse and human genes called Tg737. Mice with defects in Tg737 die shortly after birth from polycystic kidney disease. We show that the primary cilia in the kidney of Tg737 mutant mice are shorter than normal. 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.
dc.language.isoen_US
dc.relation<a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&list_uids=11062270&dopt=Abstract">Link to Article in PubMed</a>
dc.subjectAmino Acid Sequence
dc.subjectAnimals
dc.subjectChlamydomonas
dc.subjectCilia
dc.subjectCloning, Molecular
dc.subjectConserved Sequence
dc.subjectFlagella
dc.subjectHumans
dc.subjectKidney
dc.subjectMeiosis
dc.subjectMice
dc.subjectMice, Knockout
dc.subjectMicroscopy, Electron, Scanning
dc.subjectMolecular Motor Proteins
dc.subjectMolecular Sequence Data
dc.subjectMutation
dc.subjectPhenotype
dc.subjectPolycystic Kidney, Autosomal Recessive
dc.subjectProtein Binding
dc.subjectProtein Subunits
dc.subjectProteins
dc.subjectProtozoan Proteins
dc.subjectRepetitive Sequences, Amino Acid
dc.subjectSequence Alignment
dc.subjectSequence Homology, Amino Acid
dc.subject*Tumor Suppressor Proteins
dc.subjectCell Biology
dc.subjectPhysiology
dc.titleChlamydomonas IFT88 and its mouse homologue, polycystic kidney disease gene tg737, are required for assembly of cilia and flagella
dc.typeJournal Article
dc.source.journaltitleThe Journal of cell biology
dc.source.volume151
dc.source.issue3
dc.identifier.legacyfulltexthttps://escholarship.umassmed.edu/cgi/viewcontent.cgi?article=1927&amp;context=oapubs&amp;unstamped=1
dc.identifier.legacycoverpagehttps://escholarship.umassmed.edu/oapubs/928
dc.identifier.contextkey579817
refterms.dateFOA2022-08-23T16:54:51Z
html.description.abstract<p>Intraflagellar transport (IFT) is a rapid movement of multi-subunit protein particles along flagellar microtubules and is required for assembly and maintenance of eukaryotic flagella. We cloned and sequenced a Chlamydomonas cDNA encoding the IFT88 subunit of the IFT particle and identified a Chlamydomonas insertional mutant that is missing this gene. The phenotype of this mutant is normal except for the complete absence of flagella. IFT88 is homologous to mouse and human genes called Tg737. Mice with defects in Tg737 die shortly after birth from polycystic kidney disease. We show that the primary cilia in the kidney of Tg737 mutant mice are shorter than normal. 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.</p>
dc.identifier.submissionpathoapubs/928
dc.contributor.departmentProgram in Molecular Medicine
dc.contributor.departmentDepartment of Cell Biology
dc.source.pages709-18


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