Ift88

Summary

Gene Symbol: Ift88
Description: intraflagellar transport 88
Alias: AW552028, Tg737, Tg737Rpw, TgN737Rpw, Ttc10, flexo, fxo, orpk, polaris, intraflagellar transport protein 88 homolog, TPR repeat protein 10, intraflagellar transport 88 homolog, recessive polycystic kidney disease protein Tg737, tetratricopeptide repeat domain 10, tetratricopeptide repeat protein 10, tgN(Imorpk)737Rpw, transgene insert site 737, insertional mutation, polycystic kidney disease
Species: mouse
Products:     Ift88

Top Publications

  1. Banizs B, Komlosi P, Bevensee M, Schwiebert E, Bell P, Yoder B. Altered pH(i) regulation and Na(+)/HCO3(-) transporter activity in choroid plexus of cilia-defective Tg737(orpk) mutant mouse. Am J Physiol Cell Physiol. 2007;292:C1409-16 pubmed
    b>Tg737(orpk) mice have defects in cilia assembly and develop hydrocephalus in the perinatal period of life...
  2. Chamberlain C, Jeong J, Guo C, Allen B, McMahon A. Notochord-derived Shh concentrates in close association with the apically positioned basal body in neural target cells and forms a dynamic gradient during neural patterning. Development. 2008;135:1097-106 pubmed publisher
    ..This study, in which we directly observe, measure, localize and modify notochord-derived Shh ligand in the context of neural patterning, provides several new insights into mechanisms of Shh morphogen action...
  3. McDermott K, Liu B, Tlsty T, Pazour G. Primary cilia regulate branching morphogenesis during mammary gland development. Curr Biol. 2010;20:731-7 pubmed publisher
    ..In mammary gland and other organs, increased canonical Wnt [12-14] and decreased Hedgehog [15, 16] signaling decrease branching morphogenesis, suggesting that Wnt and Hedgehog signaling connect ciliary dysfunction to branching defects. ..
  4. Ocbina P, Eggenschwiler J, Moskowitz I, Anderson K. Complex interactions between genes controlling trafficking in primary cilia. Nat Genet. 2011;43:547-53 pubmed publisher
  5. Taulman P, Haycraft C, Balkovetz D, Yoder B. Polaris, a protein involved in left-right axis patterning, localizes to basal bodies and cilia. Mol Biol Cell. 2001;12:589-99 pubmed
    ..the cell population expressing Tg737 and determined the subcellular localization of its protein product called Polaris. Tg737 expression is associated with cells possessing either motile or immotile cilia and sperm...
  6. Tran P, Haycraft C, Besschetnova T, Turbe Doan A, Stottmann R, Herron B, et al. THM1 negatively modulates mouse sonic hedgehog signal transduction and affects retrograde intraflagellar transport in cilia. Nat Genet. 2008;40:403-410 pubmed publisher
    ..aln-mutant cilia have bulb-like structures at their tips in which IFT proteins (such as IFT88) are sequestered, characteristic of Chlamydomonas reinhardtii and Caenorhabditis elegans retrograde IFT mutants...
  7. Yoder B, Hou X, Guay Woodford L. The polycystic kidney disease proteins, polycystin-1, polycystin-2, polaris, and cystin, are co-localized in renal cilia. J Am Soc Nephrol. 2002;13:2508-16 pubmed
    ..elegans, the protein orthologues of the PKD-related proteins, polycystin-1 (LOV-1), polycystin-2 (PKD2), and polaris (OSM-5), co-localize in the cilia of male-specific sensory neurons, and defects in these proteins cause ..
  8. Chizhikov V, Davenport J, Zhang Q, Shih E, Cabello O, Fuchs J, et al. Cilia proteins control cerebellar morphogenesis by promoting expansion of the granule progenitor pool. J Neurosci. 2007;27:9780-9 pubmed
    ..for cilia formation and maintenance, IFT88, (also known as polaris or Tg737), which encodes intraflagellar transport 88 homolog, and Kif3a, which encodes kinesin family member 3a...
  9. Moyer J, Lee Tischler M, Kwon H, Schrick J, Avner E, Sweeney W, et al. Candidate gene associated with a mutation causing recessive polycystic kidney disease in mice. Science. 1994;264:1329-33 pubmed
    ..A complementary DNA derived from this gene predicted a peptide containing a motif that was originally identified in several genes involved in cell cycle control...

More Information

Publications72

  1. Yoder B, Tousson A, Millican L, Wu J, Bugg C, Schafer J, et al. Polaris, a protein disrupted in orpk mutant mice, is required for assembly of renal cilium. Am J Physiol Renal Physiol. 2002;282:F541-52 pubmed
    Cilia are organelles that play diverse roles, from fluid movement to sensory reception. Polaris, a protein associated with cystic kidney disease in Tg737(o)(rpk) mice, functions in a ciliogenic pathway...
  2. Han Y, Spassky N, Romaguera Ros M, Garcia Verdugo J, Aguilar A, Schneider Maunoury S, et al. Hedgehog signaling and primary cilia are required for the formation of adult neural stem cells. Nat Neurosci. 2008;11:277-84 pubmed publisher
    ..We conclude that Shh signaling, acting through the primary cilia, has a critical role in the expansion and establishment of postnatal hippocampal progenitors. ..
  3. Delaval B, Bright A, Lawson N, Doxsey S. The cilia protein IFT88 is required for spindle orientation in mitosis. Nat Cell Biol. 2011;13:461-8 pubmed publisher
    ..Here, we show that IFT88 depletion induces mitotic defects in human cultured cells, in kidney cells from the IFT88 mouse mutant Tg737(orpk) ..
  4. Schneider L, Clement C, Teilmann S, Pazour G, Hoffmann E, Satir P, et al. PDGFRalphaalpha signaling is regulated through the primary cilium in fibroblasts. Curr Biol. 2005;15:1861-6 pubmed
    ..Fibroblasts derived from Tg737(orpk) mutants fail to form normal cilia and to upregulate the level of PDGFRalpha; PDGF-AA fails to activate ..
  5. Follit J, San Agustin J, Xu F, Jonassen J, Samtani R, Lo C, et al. The Golgin GMAP210/TRIP11 anchors IFT20 to the Golgi complex. PLoS Genet. 2008;4:e1000315 pubmed publisher
    ..This work suggests that GMAP210 and IFT20 function together at the Golgi in the sorting or transport of proteins destined for the ciliary membrane. ..
  6. Clement C, Kristensen S, Møllgård K, Pazour G, Yoder B, Larsen L, et al. The primary cilium coordinates early cardiogenesis and hedgehog signaling in cardiomyocyte differentiation. J Cell Sci. 2009;122:3070-82 pubmed publisher
    ..Knockdown of the primary cilium by Ift88 and Ift20 siRNA or treatment with cyclopamine, an inhibitor of Smoothened, blocks hedgehog signaling in P19...
  7. Corbit K, Shyer A, Dowdle W, Gaulden J, Singla V, Chen M, et al. Kif3a constrains beta-catenin-dependent Wnt signalling through dual ciliary and non-ciliary mechanisms. Nat Cell Biol. 2008;10:70-6 pubmed
    ..Using three separate mutations that disrupt ciliogenesis (affecting Kif3a, Ift88 and Ofd1), we show in this study that the primary cilium restricts the activity of the canonical Wnt pathway in ..
  8. Han Y, Kim H, Dlugosz A, Ellison D, Gilbertson R, Alvarez Buylla A. Dual and opposing roles of primary cilia in medulloblastoma development. Nat Med. 2009;15:1062-5 pubmed publisher
    ..Primary cilia could serve as a diagnostic tool and provide new insights into the mechanism of tumorigenesis. ..
  9. Yoder B, Richards W, Sommardahl C, Sweeney W, Michaud E, Wilkinson J, et al. Differential rescue of the renal and hepatic disease in an autosomal recessive polycystic kidney disease mouse mutant. A new model to study the liver lesion. Am J Pathol. 1997;150:2231-41 pubmed
    ..This model was generated by differentially rescuing the renal pathology in the orpk mutant mouse that displays a hepatorenal pathology that is similar to that seen in human patients with ARPKD...
  10. Sedmak T, Wolfrum U. Intraflagellar transport molecules in ciliary and nonciliary cells of the retina. J Cell Biol. 2010;189:171-86 pubmed publisher
    ..Collectively, we provide evidence to implicate the differential composition of IFT systems in cells with and without primary cilia, thereby supporting new functions for IFT beyond its well-established role in cilia. ..
  11. Haycraft C, Banizs B, Aydin Son Y, Zhang Q, Michaud E, Yoder B. Gli2 and Gli3 localize to cilia and require the intraflagellar transport protein polaris for processing and function. PLoS Genet. 2005;1:e53 pubmed
    ..Here we provide insights into the mechanism by which defects in an IFT protein, Tg737/Polaris, affect Shh signaling in the murine limb bud...
  12. Banizs B, Pike M, Millican C, Ferguson W, Komlosi P, Sheetz J, et al. Dysfunctional cilia lead to altered ependyma and choroid plexus function, and result in the formation of hydrocephalus. Development. 2005;132:5329-39 pubmed
    ..Mutations in IFT genes, such as Tg737, result in severe developmental defects and disease...
  13. Kodani A, Salomé Sirerol Piquer M, Seol A, Garcia Verdugo J, Reiter J. Kif3a interacts with Dynactin subunit p150 Glued to organize centriole subdistal appendages. EMBO J. 2013;32:597-607 pubmed publisher
    ..Comparison to cells lacking Ift88 reveals that the centriolar functions of Kif3a are independent of IFT...
  14. Pazour G, Dickert B, Vucica Y, Seeley E, Rosenbaum J, Witman G, et al. Chlamydomonas IFT88 and its mouse homologue, polycystic kidney disease gene tg737, are required for assembly of cilia and flagella. J Cell Biol. 2000;151:709-18 pubmed
    ..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...
  15. Haycraft C, Swoboda P, Taulman P, Thomas J, Yoder B. The C. elegans homolog of the murine cystic kidney disease gene Tg737 functions in a ciliogenic pathway and is disrupted in osm-5 mutant worms. Development. 2001;128:1493-505 pubmed
    ..1 gene. osm-5 encodes a tetratricopeptide repeat (TPR)-containing protein that is the homolog of murine polaris (Tg737), a protein associated with cystic kidney disease and left-right axis patterning defects in the mouse...
  16. Zhang Q, Murcia N, Chittenden L, Richards W, Michaud E, Woychik R, et al. Loss of the Tg737 protein results in skeletal patterning defects. Dev Dyn. 2003;227:78-90 pubmed
    b>Tg737 mutant mice exhibit pathologic conditions in numerous tissues along with skeletal patterning defects...
  17. Pazour G, Baker S, Deane J, Cole D, Dickert B, Rosenbaum J, et al. The intraflagellar transport protein, IFT88, is essential for vertebrate photoreceptor assembly and maintenance. J Cell Biol. 2002;157:103-13 pubmed
    ..We further find that mice with a mutation in the IFT particle protein gene, Tg737/IFT88, have abnormal OS development and retinal degeneration...
  18. Brown N, Murcia N. Delayed cystogenesis and increased ciliogenesis associated with the re-expression of polaris in Tg737 mutant mice. Kidney Int. 2003;63:1220-9 pubmed
    ..characterized the progression of cystic disease and cilia expression in orpk, orpk;Tg737Rsq (orpk rescue), and Tg737 Delta 2-3 beta Gal;Tg737Rsq (KO rescue) mice. Methods...
  19. Keady B, Samtani R, Tobita K, Tsuchya M, San Agustin J, Follit J, et al. IFT25 links the signal-dependent movement of Hedgehog components to intraflagellar transport. Dev Cell. 2012;22:940-51 pubmed publisher
    ..Thus, IFT function is not restricted to building cilia where signaling occurs, but also plays a separable role in signal transduction events. ..
  20. Jones C, Roper V, Foucher I, Qian D, Banizs B, Petit C, et al. Ciliary proteins link basal body polarization to planar cell polarity regulation. Nat Genet. 2008;40:69-77 pubmed
    ..Here, we show that a gene encoding a ciliary protein (a 'ciliary gene'), Ift88, also known as Polaris, is required for establishing epithelial PCP and for convergent extension of the cochlear duct of Mus musculus...
  21. Nauli S, Kawanabe Y, Kaminski J, Pearce W, Ingber D, Zhou J. Endothelial cilia are fluid shear sensors that regulate calcium signaling and nitric oxide production through polycystin-1. Circulation. 2008;117:1161-71 pubmed publisher
    ..Cells with the Pkd1(null/null) or Tg737(orpk/orpk) mutation encoded for polycystin-1 or polaris, respectively, are unable to transmit extracellular shear stress into intracellular calcium signaling and ..
  22. Cano D, Murcia N, Pazour G, Hebrok M. Orpk mouse model of polycystic kidney disease reveals essential role of primary cilia in pancreatic tissue organization. Development. 2004;131:3457-67 pubmed
    ..Here we show that in orpk mice, a model system for PKD that harbors a mutation in the gene that encodes the polaris protein, pancreatic defects start to occur at the end of gestation, with an initial expansion of the developing ..
  23. Johnson E, Nicola T, Roarty K, Yoder B, Haycraft C, Serra R. Role for primary cilia in the regulation of mouse ovarian function. Dev Dyn. 2008;237:2053-60 pubmed publisher
    b>Ift88 is a component of the intraflagellar transport complex required for formation and maintenance of cilia. Disruption of Ift88 results in depletion of cilia...
  24. Higginbotham H, Eom T, Mariani L, Bachleda A, Hirt J, Gukassyan V, et al. Arl13b in primary cilia regulates the migration and placement of interneurons in the developing cerebral cortex. Dev Cell. 2012;23:925-38 pubmed publisher
  25. Perrone C, Tritschler D, Taulman P, Bower R, Yoder B, Porter M. A novel dynein light intermediate chain colocalizes with the retrograde motor for intraflagellar transport at sites of axoneme assembly in chlamydomonas and Mammalian cells. Mol Biol Cell. 2003;14:2041-56 pubmed
  26. Baker S, Freeman K, Luby Phelps K, Pazour G, Besharse J. IFT20 links kinesin II with a mammalian intraflagellar transport complex that is conserved in motile flagella and sensory cilia. J Biol Chem. 2003;278:34211-8 pubmed
    ..sensory cilia of photo-receptors, we investigated protein interactions among four mammalian IFT proteins: IFT88/Polaris, IFT57/Hippi, IFT52/NGD5, and IFT20...
  27. Zhang Q, Davenport J, Croyle M, Haycraft C, Yoder B. Disruption of IFT results in both exocrine and endocrine abnormalities in the pancreas of Tg737(orpk) mutant mice. Lab Invest. 2005;85:45-64 pubmed
    ..Here, we further establish a connection between cilia dysfunction and disease by showing that loss of polaris (Tg737), an intraflagellar transport (IFT) protein required for ciliogenesis, causes abnormalities in the ..
  28. Pazour G, San Agustin J, Follit J, Rosenbaum J, Witman G. Polycystin-2 localizes to kidney cilia and the ciliary level is elevated in orpk mice with polycystic kidney disease. Curr Biol. 2002;12:R378-80 pubmed
  29. Nauli S, Alenghat F, Luo Y, Williams E, Vassilev P, Li X, et al. Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells. Nat Genet. 2003;33:129-37 pubmed
    ..Furthermore, cilia are malformed in mice with PKD with mutations in TgN737Rpw (encoding polaris)...
  30. Follit J, Tuft R, Fogarty K, Pazour G. The intraflagellar transport protein IFT20 is associated with the Golgi complex and is required for cilia assembly. Mol Biol Cell. 2006;17:3781-92 pubmed
    ..This work suggests that IFT20 functions in the delivery of ciliary membrane proteins from the Golgi complex to the cilium. ..
  31. Follit J, Xu F, Keady B, Pazour G. Characterization of mouse IFT complex B. Cell Motil Cytoskeleton. 2009;66:457-68 pubmed publisher
    ..All of the proteins except IFT172 immunoprecipitate IFT88 indicating that they are co-assembled into a complex...
  32. Haycraft C, Zhang Q, Song B, Jackson W, Detloff P, Serra R, et al. Intraflagellar transport is essential for endochondral bone formation. Development. 2007;134:307-16 pubmed
    ..the role of cilia during limb development, we generated a conditional allele of the IFT protein Ift88 (polaris). Using the Cre-lox system, we disrupted cilia on different cell populations within the developing limb...
  33. Liem K, Ashe A, He M, Satir P, Moran J, Beier D, et al. The IFT-A complex regulates Shh signaling through cilia structure and membrane protein trafficking. J Cell Biol. 2012;197:789-800 pubmed publisher
  34. Hou Y, Qin H, Follit J, Pazour G, Rosenbaum J, Witman G. Functional analysis of an individual IFT protein: IFT46 is required for transport of outer dynein arms into flagella. J Cell Biol. 2007;176:653-65 pubmed
    ..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. ..
  35. Insinna C, Humby M, Sedmak T, Wolfrum U, Besharse J. Different roles for KIF17 and kinesin II in photoreceptor development and maintenance. Dev Dyn. 2009;238:2211-22 pubmed publisher
    ..In contrast, dominant-negative KIF17 has no obvious effect on inner segment or synaptic organization but has an immediate impact on outer segment assembly. ..
  36. Wong S, Seol A, So P, Ermilov A, Bichakjian C, Epstein E, et al. Primary cilia can both mediate and suppress Hedgehog pathway-dependent tumorigenesis. Nat Med. 2009;15:1055-61 pubmed publisher
    ..and we test the role of cilia in BCC by conditionally deleting Kif3a (encoding kinesin family member 3A) or Ift88 (encoding intraflagellar transport protein 88), genes required for ciliogenesis, in two Hh pathway-dependent mouse ..
  37. Friedland Little J, Hoffmann A, Ocbina P, Peterson M, Bosman J, Chen Y, et al. A novel murine allele of Intraflagellar Transport Protein 172 causes a syndrome including VACTERL-like features with hydrocephalus. Hum Mol Genet. 2011;20:3725-37 pubmed publisher
    ..Truncated Ift172 mutant cilia showed defects of the distal ciliary axoneme, including disrupted IFT88 localization and Hh-dependent Gli2 localization...
  38. Liu A, Wang B, Niswander L. Mouse intraflagellar transport proteins regulate both the activator and repressor functions of Gli transcription factors. Development. 2005;132:3103-11 pubmed
    ..Here we report that homozygous mutants for flexo (Fxo), a hypomorphic allele of mouse IFT88 generated in our ENU mutagenesis screen, exhibit polydactyly in all ..
  39. Huangfu D, Liu A, Rakeman A, Murcia N, Niswander L, Anderson K. Hedgehog signalling in the mouse requires intraflagellar transport proteins. Nature. 2003;426:83-7 pubmed
    ..is an allele of the previously uncharacterized mouse homologue of IFT172; and fxo is a new hypomorphic allele of polaris, the mouse homologue of IFT88...
  40. Murcia N, Richards W, Yoder B, Mucenski M, Dunlap J, Woychik R. The Oak Ridge Polycystic Kidney (orpk) disease gene is required for left-right axis determination. Development. 2000;127:2347-55 pubmed
    ..We have named the product of the Tg737 gene Polaris, which is based on the various polarity related defects associated with the different alleles of the Tg737 gene.
  41. Sas K, Janech M, Favre E, Arthur J, Bell P. Cilia movement regulates expression of the Raf-1 kinase inhibitor protein. Am J Physiol Renal Physiol. 2011;300:F1163-70 pubmed publisher
    ..In terms of polycystic kidney disease, loss of cilia and therefore sensitivity to flow may lead to reduced RKIP levels, activation of the MAPK pathway, and contribute to the formation of cysts. ..
  42. Cela P, Hampl M, Shylo N, Christopher K, Kavková M, Landová M, et al. Ciliopathy Protein Tmem107 Plays Multiple Roles in Craniofacial Development. J Dent Res. 2018;97:108-117 pubmed publisher
    ..cilia revealed region-specific changes in ciliary morphology accompanied by alteration of acetylated tubulin and IFT88 expression...
  43. Rix S, Calmont A, Scambler P, Beales P. An Ift80 mouse model of short rib polydactyly syndromes shows defects in hedgehog signalling without loss or malformation of cilia. Hum Mol Genet. 2011;20:1306-14 pubmed publisher
    ..These data suggest that there is an absolute requirement for Ift80 in hedgehog signalling, but low level expression permits ciliogenesis indicating separate but linked roles for this protein in formation and function. ..
  44. Ye X, Zeng H, Ning G, Reiter J, Liu A. C2cd3 is critical for centriolar distal appendage assembly and ciliary vesicle docking in mammals. Proc Natl Acad Sci U S A. 2014;111:2164-9 pubmed publisher
    ..C2cd3 is also required for recruiting the intraflagellar transport proteins Ift88 and Ift52 to the mother centriole...
  45. Pampliega O, Orhon I, Patel B, Sridhar S, Díaz Carretero A, Beau I, et al. Functional interaction between autophagy and ciliogenesis. Nature. 2013;502:194-200 pubmed publisher
    ..We propose that basal autophagy regulates ciliary growth through the degradation of proteins required for intraflagellar transport. Compromised ability to activate the autophagic response may underlie some common ciliopathies. ..
  46. Berbari N, Pasek R, Malarkey E, Yazdi S, McNair A, Lewis W, et al. Leptin resistance is a secondary consequence of the obesity in ciliopathy mutant mice. Proc Natl Acad Sci U S A. 2013;110:7796-801 pubmed publisher
    ..In contrast to the current dogma, a longitudinal study of conditional Ift88 cilia mutant mice under different states of adiposity indicates that leptin resistance is present only when ..
  47. Kaushik A, Martin J, Zhang Q, Sheffield V, Morcuende J. Cartilage abnormalities associated with defects of chondrocytic primary cilia in Bardet-Biedl syndrome mutant mice. J Orthop Res. 2009;27:1093-9 pubmed publisher
    ..These data indicate that Bbs genes and their functions in the chondrocytic primary cilium are important for normal articular cartilage maintenance. ..
  48. Lehman J, Michaud E, Schoeb T, Aydin Son Y, Miller M, Yoder B. The Oak Ridge Polycystic Kidney mouse: modeling ciliopathies of mice and men. Dev Dyn. 2008;237:1960-71 pubmed publisher
    ..The ORPK mouse arose through integration of a transgene into an intron of the Ift88 gene resulting in a hypomorphic allele (Ift88Tg737Rpw)...
  49. Mahaffey J, Grego Bessa J, Liem K, Anderson K. Cofilin and Vangl2 cooperate in the initiation of planar cell polarity in the mouse embryo. Development. 2013;140:1262-71 pubmed publisher
    ..We propose that Vangl2 and cofilin cooperate to target Rab11(+) vesicles containing PCP proteins to the apical membrane during the initiation of planar cell polarity. ..
  50. Song B, Haycraft C, Seo H, Yoder B, Serra R. Development of the post-natal growth plate requires intraflagellar transport proteins. Dev Biol. 2007;305:202-16 pubmed
    ..The results suggest a model in which IFT/cilia act to maintain the columnar organization of the growth plate via the process of chondrocyte rotation. ..
  51. Wei Q, Zhang Y, Li Y, Zhang Q, Ling K, Hu J. The BBSome controls IFT assembly and turnaround in cilia. Nat Cell Biol. 2012;14:950-7 pubmed publisher
    ..Our results identify the BBSome as the key player regulating IFT assembly and turnaround in cilia...
  52. Tong C, Han Y, Shah J, Obernier K, Guinto C, Alvarez Buylla A. Primary cilia are required in a unique subpopulation of neural progenitors. Proc Natl Acad Sci U S A. 2014;111:12438-43 pubmed publisher
    ..genes essential for intraflagellar transport [kinesin family member 3A (Kif3a) and intraflagellar transport 88 (Ift88)] and Cre drivers that are activated at early [Nestin; embryonic day 10.5 (E10...
  53. Osborn D, Roccasecca R, McMurray F, Hernandez Hernandez V, Mukherjee S, Barroso I, et al. Loss of FTO antagonises Wnt signaling and leads to developmental defects associated with ciliopathies. PLoS ONE. 2014;9:e87662 pubmed publisher
    ..Furthermore, we present the first evidence that FTO plays a role in development and cilia formation/function. ..
  54. Wann A, Zuo N, Haycraft C, Jensen C, Poole C, McGlashan S, et al. Primary cilia mediate mechanotransduction through control of ATP-induced Ca2+ signaling in compressed chondrocytes. FASEB J. 2012;26:1663-71 pubmed publisher
    ..We generated conditionally immortalized wild-type (WT) and IFT88(orpk) (ORPK) mutant chondrocytes that lack primary cilia and assessed intracellular Ca(2+) signaling, ..
  55. Colvin J, Feldman B, Nadeau J, Goldfarb M, Ornitz D. Genomic organization and embryonic expression of the mouse fibroblast growth factor 9 gene. Dev Dyn. 1999;216:72-88 pubmed
    ..Fgf9 is coexpressed with other Fgf genes in some skeletal myoblasts, in limb apical ectoderm, in craniofacial ectoderm, and in the retina, inner ear, and tooth bud. Dev Dyn 1999;216:72-88. ..
  56. Chacon Heszele M, Ren D, Reynolds A, Chi F, Chen P. Regulation of cochlear convergent extension by the vertebrate planar cell polarity pathway is dependent on p120-catenin. Development. 2012;139:968-78 pubmed publisher
    ..Together, these results indicate that the vertebrate PCP pathway regulates CE and hair cell polarity independently and that a p120-catenin-dependent mechanism regulates CE of the cochlea. ..
  57. Smith S, Qu H, Taleb N, Kishimoto N, Scheel D, Lu Y, et al. Rfx6 directs islet formation and insulin production in mice and humans. Nature. 2010;463:775-80 pubmed publisher
    ..These studies demonstrate a unique position for Rfx6 in the hierarchy of factors that coordinate pancreatic islet development in both mice and humans. Rfx6 could prove useful in efforts to generate beta-cells for patients with diabetes. ..
  58. Sawamoto K, Wichterle H, Gonzalez Perez O, Cholfin J, Yamada M, Spassky N, et al. New neurons follow the flow of cerebrospinal fluid in the adult brain. Science. 2006;311:629-32 pubmed
    ..Results suggest that polarized epithelial cells contribute important vectorial information for guidance of young, migrating neurons. ..
  59. Foerster P, Daclin M, Asm S, Faucourt M, Boletta A, Genovesio A, et al. mTORC1 signaling and primary cilia are required for brain ventricle morphogenesis. Development. 2017;144:201-210 pubmed publisher
    ..These results suggest that primary cilia regulate ventricle morphogenesis by acting as a brake on the mTORC1 pathway. This opens new avenues for the diagnosis and treatment of hydrocephalus. ..
  60. Omori Y, Chaya T, Katoh K, Kajimura N, Sato S, Muraoka K, et al. Negative regulation of ciliary length by ciliary male germ cell-associated kinase (Mak) is required for retinal photoreceptor survival. Proc Natl Acad Sci U S A. 2010;107:22671-6 pubmed publisher
    ..We observed accumulation of intraflagellar transport 88 (IFT88) and IFT57, expansion of kinesin family member 3A (Kif3a), and acetylated ?-tubulin signals in the Mak-null ..
  61. Gazea M, Tasouri E, Tolve M, Bosch V, Kabanova A, Gojak C, et al. Primary cilia are critical for Sonic hedgehog-mediated dopaminergic neurogenesis in the embryonic midbrain. Dev Biol. 2016;409:55-71 pubmed publisher
    ..We analyzed mutant mouse lines deficient in the intraflagellar transport protein IFT88, which is critical for primary cilia function. Conditional inactivation of Ift88 in the midbrain after E9...
  62. Schock E, Struve J, Chang C, Williams T, Snedeker J, Attia A, et al. A tissue-specific role for intraflagellar transport genes during craniofacial development. PLoS ONE. 2017;12:e0174206 pubmed publisher
    ..during craniofacial development we conditionally deleted three separate intraflagellar transport genes, Kif3a, Ift88 and Ttc21b with three distinct drivers, Wnt1-Cre, Crect and AP2-Cre which drive recombination in neural crest, ..
  63. Bell P, Fitzgibbon W, Sas K, Stenbit A, Amria M, Houston A, et al. Loss of primary cilia upregulates renal hypertrophic signaling and promotes cystogenesis. J Am Soc Nephrol. 2011;22:839-48 pubmed publisher
    ..Conditional knockout of the primary cilia ift88 gene leads to delayed, adult-onset renal cystic disease, which provides a window of opportunity to conduct ..