title: Nephrocystins and MKS proteins interact with IFT particle and facilitate transport of selected ciliary cargos.
abstract: Cilia are required for the development and function of many organs. Efficient transport of protein cargo along ciliary axoneme is necessary to sustain these processes. Despite its importance, the mode of interaction between the intraflagellar ciliary transport (IFT) mechanism and its cargo proteins remains poorly understood. Our studies demonstrate that IFT particle components, and a Meckel-Gruber syndrome 1 (MKS1)-related, B9 domain protein, B9d2, bind each other and contribute to the ciliary localization of Inversin (Nephrocystin 2). B9d2, Inversin, and Nephrocystin 5 support, in turn, the transport of a cargo protein, Opsin, but not another photoreceptor ciliary transmembrane protein, Peripherin. Interestingly, the components of this mechanism also contribute to the formation of planar cell polarity in mechanosensory epithelia. These studies reveal a molecular mechanism that mediates the transport of selected ciliary cargos and is of fundamental importance for the differentiation and survival of sensory cells.
authors: Division of Craniofacial and Molecular Genetics, and Program in Genetics, Sackler School of Graduate Biomedical Studies, Tufts University, Boston, MA, USA.
authors: Chengtian
authors: C
authors: Zhao
authors: Jarema
authors: J
authors: Malicki
references: Mech Dev. 2007 Aug;124(7-8):605-16
references: 17576052
references: Cell. 2000 Jul 21;102(2):175-87
references: 10943838
references: Am J Kidney Dis. 2007 Nov;50(5):855-64
references: 17954299
references: Proc Natl Acad Sci U S A. 2008 Feb 26;105(8):2853-8
references: 18287022
references: Pediatr Nephrol. 2009 Dec;24(12):2333-44
references: 18607645
references: Nature. 2005 Jul 28;436(7050):583-7
references: 16049494
references: Hum Genet. 2002 Apr;110(4):377-84
references: 11941489
references: J Am Soc Nephrol. 2004 Jul;15(7):1744-55
references: 15213262
references: Ophthalmic Genet. 2007 Sep;28(3):113-25
references: 17896309
references: J Cell Sci. 2008 Jun 1;121(11):1907-15
references: 18492793
references: Nat Genet. 2005 Oct;37(10):1135-40
references: 16170314
references: Vision Res. 2007 Dec;47(27):3394-407
references: 18022666
references: Mech Dev. 2002 Jan;110(1-2):125-38
references: 11744374
references: Development. 2004 Aug;131(16):4085-93
references: 15269167
references: Methods Cell Biol. 2004;76:333-84
references: 15602883
references: Proc Natl Acad Sci U S A. 2010 Jan 26;107(4):1488-93
references: 20080638
references: Chem Senses. 2009 Jun;34(5):451-64
references: 19406873
references: Trends Cell Biol. 2009 Jul;19(7):306-16
references: 19560357
references: Cell. 2010 Jun 25;141(7):1208-19
references: 20603001
references: Mol Biol Cell. 2010 Aug 1;21(15):2696-706
references: 20534810
references: Cell. 2007 Jun 15;129(6):1201-13
references: 17574030
references: Mol Biol Cell. 2004 Apr;15(4):2027-37
references: 14767063
references: Curr Top Dev Biol. 2008;85:23-61
references: 19147001
references: Nat Genet. 2010 Jul;42(7):619-25
references: 20512146
references: Development. 2004 Sep;131(18):4467-76
references: 15306567
references: Nat Rev Mol Cell Biol. 2002 Nov;3(11):813-25
references: 12415299
references: Nat Genet. 2003 Aug;34(4):413-20
references: 12872123
references: Nat Genet. 1998 Oct;20(2):149-56
references: 9771707
references: Nat Genet. 2008 Jan;40(1):69-77
references: 18066062
references: Dev Dyn. 2007 Nov;236(11):3088-99
references: 17937395
references: Hum Mol Genet. 2005 Mar 1;14(5):645-56
references: 15661758
references: J Biol Chem. 2003 Sep 5;278(36):34211-8
references: 12821668
references: Nat Cell Biol. 2002 Aug;4(8):610-5
references: 12105418
references: J Cell Sci. 2009 Mar 1;122(Pt 5):611-24
references: 19208769
references: Mol Biol Cell. 2002 Sep;13(9):3096-106
references: 12221118
references: J Cell Biol. 2004 Jan 19;164(2):255-66
references: 14718520
references: Invest Ophthalmol Vis Sci. 2001 Mar;42(3):589-600
references: 11222515
references: J Am Soc Nephrol. 2009 Jan;20(1):23-35
references: 19118152
references: Development. 2003 May;130(9):1725-34
references: 12642479
references: Development. 1999 Jun;126(14):3253-62
references: 10375514
references: Nat Cell Biol. 2008 Apr;10(4):437-44
references: 18364699
references: Hum Mutat. 2005 Apr;25(4):411
references: 15776426
references: Curr Biol. 2006 May 23;16(10):945-57
references: 16713951
references: Nat Genet. 2008 Apr;40(4):443-8
references: 18327255
references: J Cell Biol. 2000 Dec 25;151(7):1369-80
references: 11134067
references: Vision Res. 2006 Dec;46(27):4427-33
references: 17010408
references: Nat Genet. 2003 Aug;34(4):455-9
references: 12872122
references: Proc Natl Acad Sci U S A. 2010 Mar 30;107(13):6052-7
references: 20231443
references: Hum Mutat. 2007 May;28(5):523-4
references: 17397051
references: Hum Mol Genet. 2002 Dec 15;11(26):3345-50
references: 12471060
references: Neuron. 2004 Jun 10;42(5):703-16
references: 15182712
references: Hum Mol Genet. 2009 Dec 1;18(23):4565-75
references: 19776033
references: Dev Dyn. 2009 Sep;238(9):2115-38
references: 19582864
references: Mol Biol Cell. 2008 May;19(5):2154-68
references: 18337471
references: Development. 2000 Jun;127(11):2347-55
references: 10804177
references: Nephrol Dial Transplant. 2006 Jul;21(7):1989-91
references: 16522655
references: Proc Natl Acad Sci U S A. 2007 Oct 2;104(40):15917-22
references: 17898177
references: Proc Natl Acad Sci U S A. 2003 Apr 1;100(7):3965-70
references: 12651948
references: Mol Biol Cell. 2007 Nov;18(11):4353-64
references: 17761526
references: Int Rev Cytol. 2000;195:215-67
references: 10603577
references: Nat Genet. 2005 May;37(5):537-43
references: 15852005