Gene Symbol: Hippi
Description: intraflagellar transport 57
Alias: 4833420A15Rik, Esrrbl1, Hippi, MHS4R2, intraflagellar transport protein 57 homolog, HIP1-interacting protein, Vestrogen-related receptor beta like 1, estrogen-related receptor beta like 1, huntingtin-interacting protein-1 protein interactor, intraflagellar transport 57 homolog
Species: mouse
Products:     Hippi

Top Publications

  1. Tsujikawa M, Malicki J. Intraflagellar transport genes are essential for differentiation and survival of vertebrate sensory neurons. Neuron. 2004;42:703-16 pubmed
    ..These studies reveal an essential role for IFT genes in vertebrate sensory neurons and implicate the molecular components of intraflagellar transport in degenerative disorders of these cells. ..
  2. Blacque O, Perens E, Boroevich K, Inglis P, Li C, Warner A, et al. Functional genomics of the cilium, a sensory organelle. Curr Biol. 2005;15:935-41 pubmed
    ..7a. Together, our findings help define a ciliary transcriptome and suggest that DYF-13, an evolutionarily conserved protein, is a novel core IFT component required for cilia function. ..
  3. 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. ..
  4. 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. ..
  5. 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
    ..of photo-receptors, we investigated protein interactions among four mammalian IFT proteins: IFT88/Polaris, IFT57/Hippi, IFT52/NGD5, and IFT20...
  6. Follit J, Xu F, Keady B, Pazour G. Characterization of mouse IFT complex B. Cell Motil Cytoskeleton. 2009;66:457-68 pubmed publisher
    ..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. ..
  7. Luby Phelps K, Fogerty J, Baker S, Pazour G, Besharse J. Spatial distribution of intraflagellar transport proteins in vertebrate photoreceptors. Vision Res. 2008;48:413-23 pubmed
    ..IFT52-GFP and IFT57-GFP mimicked this pattern in transgenic Xenopus. ..
  8. Ferrier V. Hip, hip, hippi!. Nat Cell Biol. 2002;4:E30 pubmed
  9. Keady B, Le Y, Pazour G. IFT20 is required for opsin trafficking and photoreceptor outer segment development. Mol Biol Cell. 2011;22:921-30 pubmed publisher
    ..Since IFT20 dynamically moves between the Golgi complex and the connecting cilium, the current work suggests that rhodopsin and opsins are cargo for IFT transport. ..

More Information


  1. 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
    ..These results suggest that Mak is essential for the regulation of ciliary length and is required for the long-term survival of photoreceptors. ..
  2. Houde C, Dickinson R, Houtzager V, Cullum R, Montpetit R, Metzler M, et al. Hippi is essential for node cilia assembly and Sonic hedgehog signaling. Dev Biol. 2006;300:523-33 pubmed
    b>Hippi functions as an adapter protein that mediates pro-apoptotic signaling from poly-glutamine-expanded huntingtin, an established cause of Huntington disease, to the extrinsic cell death pathway...
  3. Jurczyk A, Gromley A, Redick S, San Agustin J, Witman G, Pazour G, et al. Pericentrin forms a complex with intraflagellar transport proteins and polycystin-2 and is required for primary cilia assembly. J Cell Biol. 2004;166:637-43 pubmed
    ..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. ..
  4. Ezratty E, Stokes N, Chai S, Shah A, Williams S, Fuchs E. A role for the primary cilium in Notch signaling and epidermal differentiation during skin development. Cell. 2011;145:1129-41 pubmed publisher
    ..These findings unveil temporally and spatially distinct functions for primary cilia at the nexus of signaling, proliferation, and differentiation. ..
  5. Datta M, Bhattacharyya N. Regulation of RE1 protein silencing transcription factor (REST) expression by HIP1 protein interactor (HIPPI). J Biol Chem. 2011;286:33759-69 pubmed publisher
    Earlier we have shown that the proapoptotic protein HIPPI (huntingtin interacting protein 1 (HIP1) protein interactor) along with its molecular partner HIP1 could regulate transcription of the caspase-1 gene...
  6. Wang C, Low W, Liu A, Wang B. Centrosomal protein DZIP1 regulates Hedgehog signaling by promoting cytoplasmic retention of transcription factor GLI3 and affecting ciliogenesis. J Biol Chem. 2013;288:29518-29 pubmed publisher
    ..Therefore, DZIP1 is the first known ciliogenic protein that regulates Hedgehog signaling through a dual mechanism and that biochemically links IFT machinery with Hedgehog pathway components. ..
  7. Ishikawa H, Ide T, Yagi T, Jiang X, Hirono M, Sasaki H, et al. TTC26/DYF13 is an intraflagellar transport protein required for transport of motility-related proteins into flagella. elife. 2014;3:e01566 pubmed
    ..These results support the concept that different IFT proteins are responsible for different cargo subsets, providing a possible explanation for the complexity of the IFT machinery. DOI: http://dx.doi.org/10.7554/eLife.01566.001...
  8. Stanton S, Blanck J, Locker J, Schreiber Agus N. Rybp interacts with Hippi and enhances Hippi-mediated apoptosis. Apoptosis. 2007;12:2197-206 pubmed
    ..Here we characterize a novel interaction between Rybp and Hippi, a protein implicated in neuronal apoptosis as well as in the pathogenesis of Huntington's disease...
  9. Gervais F, Singaraja R, Xanthoudakis S, Gutekunst C, Leavitt B, Metzler M, et al. Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi. Nat Cell Biol. 2002;4:95-105 pubmed
    ..Free Hip-1 binds to a hitherto unknown polypeptide, Hippi (Hip-1 protein interactor), which has partial sequence homology to Hip-1 and similar tissue and subcellular ..
  10. Seo S, Zhang Q, Bugge K, Breslow D, Searby C, Nachury M, et al. A novel protein LZTFL1 regulates ciliary trafficking of the BBSome and Smoothened. PLoS Genet. 2011;7:e1002358 pubmed publisher
    ..Finally, we found that BBS proteins and LZTFL1 regulate ciliary trafficking of hedgehog signal transducer, Smoothened. Our findings suggest that LZTFL1 is an important regulator of BBSome ciliary trafficking and hedgehog signaling. ..
  11. Liew G, Ye F, Nager A, Murphy J, Lee J, Aguiar M, et al. The intraflagellar transport protein IFT27 promotes BBSome exit from cilia through the GTPase ARL6/BBS3. Dev Cell. 2014;31:265-78 pubmed publisher
    ..Thus, we propose that IFT27 separates from IFT-B inside cilia to promote ARL6 activation, BBSome coat assembly, and subsequent ciliary exit, mirroring the process by which BBSome mediates cargo entry into cilia. ..
  12. 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...
  13. Howard P, Jue S, Maurer R. Interaction of mouse TTC30/DYF-1 with multiple intraflagellar transport complex B proteins and KIF17. Exp Cell Res. 2013;319:2275-81 pubmed publisher