Reactome | Recruitment of transition zone proteins

Recruitment of transition zone proteins

Stable Identifier

R-HSA-5626681

Type

Reaction [binding]

Species

Homo sapiens

Compartment

cytosol

ReviewStatus

5/5

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The transition zone is a protein-rich zone at the base of the cilium that forms after maturation of the mother centriole and prior to or concurrent with the initiation of intraflagellar transport (IFT) (reviewed in Benzing and Schermer, 2011; Reiter et al, 2012). The transition zone consists of a growing number of proteins and protein complexes, many of whose genes are associated with ciliopathies such as nephronophthisis, Meckel-Gruber syndrome and Joubert's syndrome (Reiter et al, 2006; Hu et al, 2010; Sang et al, 2011; Williams et al, 2011; Garcia-Gonzalo et al, 2011; Chih et al, 2012; reviewed in Reiter et al, 2012). In conjunction with SEPT2, which was recently shown to form a septin ring diffusion barrier at the base of the cilium, the transition zone and its resident proteins contribute to protein sorting and ciliary membrane composition and act as a ciliary gate (Hu et al, 2010; Williams et al, 2011; Garcia-Gonzalo et al, 2011; Chih et al, 2012; reviewed in Reiter et al, 2012).

Literature References

PubMed ID	Title	Journal	Year
16357211	Tectonic, a novel regulator of the Hedgehog pathway required for both activation and inhibition Reiter, JF, Skarnes, WC	Genes Dev.	2006
22653444	The base of the cilium: roles for transition fibres and the transition zone in ciliary formation, maintenance and compartmentalization Reiter, JF, Blacque, OE, Leroux, MR	EMBO Rep.	2012
21725307	A transition zone complex regulates mammalian ciliogenesis and ciliary membrane composition Garcia-Gonzalo, FR, Corbit, KC, Sirerol-Piquer, MS, Ramaswami, G, Otto, EA, Noriega, TR, Seol, AD, Robinson, JF, Bennett, CL, Josifova, DJ, García-Verdugo, JM, Katsanis, N, Hildebrandt, F, Reiter, JF	Nat. Genet.	2011
21565611	Mapping the NPHP-JBTS-MKS protein network reveals ciliopathy disease genes and pathways Sang, L, Miller, JJ, Corbit, KC, Giles, RH, Brauer, MJ, Otto, EA, Baye, LM, Wen, X, Scales, SJ, Kwong, M, Huntzicker, EG, Sfakianos, MK, Sandoval, W, Bazan, JF, Kulkarni, P, Garcia-Gonzalo, FR, Seol, AD, O'Toole, JF, Held, S, Reutter, HM, Lane, WS, Rafiq, MA, Noor, A, Ansar, M, Devi, AR, Sheffield, VC, Slusarski, DC, Vincent, JB, Doherty, DA, Hildebrandt, F, Reiter, JF, Jackson, PK	Cell	2011
21792235	Transition zone proteins and cilia dynamics Benzing, T, Schermer, B	Nat. Genet.	2011
20558667	A septin diffusion barrier at the base of the primary cilium maintains ciliary membrane protein distribution Hu, Q, Milenkovic, L, Jin, H, Scott, MP, Nachury, MV, Spiliotis, ET, Nelson, WJ	Science	2010
21422230	MKS and NPHP modules cooperate to establish basal body/transition zone membrane associations and ciliary gate function during ciliogenesis Williams, CL, Li, C, Kida, K, Inglis, PN, Mohan, S, Semenec, L, Bialas, NJ, Stupay, RM, Chen, N, Blacque, OE, Yoder, BK, Leroux, MR	J. Cell Biol.	2011
22179047	A ciliopathy complex at the transition zone protects the cilia as a privileged membrane domain Chih, B, Liu, P, Chinn, Y, Chalouni, C, Komuves, LG, Hass, PE, Sandoval, W, Peterson, AS	Nat. Cell Biol.	2012