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| | DHH | Hedgehog protein; Intercellular signal essential for a variety of patterning events during development. (396 aa) |
| | DISP2 | Dispatched RND transporter family member 2. (1403 aa) |
| | INPP5E | Inositol polyphosphate-5-phosphatase E. (656 aa) |
| | LZTFL1 | Leucine zipper transcription factor-like protein 1; Regulates ciliary localization of the BBSome complex. Together with the BBSome complex, controls SMO ciliary trafficking and contributes to the sonic hedgehog (SHH) pathway regulation. May play a role in neurite outgrowth. May have tumor suppressor function (By similarity). (299 aa) |
| | TMEM107 | Transmembrane protein 107. (140 aa) |
| | TANC2 | Tetratricopeptide repeat, ankyrin repeat and coiled-coil containing 2. (1989 aa) |
| | EVC2 | Limbin; Component of the EvC complex that positively regulates ciliary Hedgehog (Hh) signaling. Plays a critical role in bone formation and skeletal development. May be involved in early embryonic morphogenesis. (1300 aa) |
| | EVC | EvC ciliary complex subunit 1. (993 aa) |
| | ARL3 | ADP-ribosylation factor-like protein 3; Small GTP-binding protein which cycles between an inactive GDP-bound and an active GTP-bound form, and the rate of cycling is regulated by guanine nucleotide exchange factors (GEF) and GTPase- activating proteins (GAP). Required for normal cytokinesis and cilia signaling. Requires assistance from GTPase-activating proteins (GAPs) like RP2 and PDE6D, in order to cycle between inactive GDP-bound and active GTP-bound forms. Required for targeting proteins to the cilium, including myristoylated NPHP3 and prenylated INPP5E. Targets NPHP3 to the ciliar [...] (198 aa) |
| | TTC21B | Tetratricopeptide repeat domain 21B. (1316 aa) |
| | RPGRIP1 | X-linked retinitis pigmentosa GTPase regulator-interacting protein 1; May function as scaffolding protein. Required for normal location of RPGR at the connecting cilium of photoreceptor cells. Required for normal disk morphogenesis and disk organization in the outer segment of photoreceptor cells and for survival of photoreceptor cells; Belongs to the RPGRIP1 family. (1213 aa) |
| | BBS7 | Bardet-Biedl syndrome 7 protein homolog; The BBSome complex is thought to function as a coat complex required for sorting of specific membrane proteins to the primary cilia. The BBSome complex is required for ciliogenesis but is dispensable for centriolar satellite function. (715 aa) |
| | ANKS6 | Ankyrin repeat and sterile alpha motif domain containing 6. (864 aa) |
| | ARL13B | ADP ribosylation factor like GTPase 13B. (429 aa) |
| | NPHP3 | Nephrocystin 3. (1331 aa) |
| | IFT57 | Intraflagellar transport protein 57 homolog; Required for the formation of cilia. Plays an indirect role in sonic hedgehog signaling, cilia being required for all activity of the hedgehog pathway. Has pro-apoptotic function via its interaction with HIP1, leading to recruit caspase-8 (CASP8) and trigger apoptosis. Has the ability to bind DNA sequence motif 5'-AAAGACATG-3' present in the promoter of caspase genes such as CASP1, CASP8 and CASP10, suggesting that it may act as a transcription regulator; however the relevance of such function remains unclear (By similarity). (429 aa) |
| | SPOP | Speckle-type POZ protein; Component of a cullin-RING-based BCR (BTB-CUL3-RBX1) E3 ubiquitin-protein ligase complex that mediates the ubiquitination of target proteins, leading most often to their proteasomal degradation. In complex with CUL3, involved in ubiquitination and proteasomal degradation of BRMS1, DAXX, PDX1/IPF1, GLI2 and GLI3. In complex with CUL3, involved in ubiquitination of MACROH2A1 and BMI1; this does not lead to their proteasomal degradation. Inhibits transcriptional activation of PDX1/IPF1 targets, such as insulin, by promoting PDX1/IPF1 degradation. The cullin-RING- [...] (374 aa) |
| | BBS9 | Uncharacterized protein. (887 aa) |
| | ANKS3 | Ankyrin repeat and sterile alpha motif domain containing 3. (655 aa) |
| | GLI1 | GLI family zinc finger 1. (1105 aa) |
| | HSPB11 | Heat shock protein family B (small) member 11. (143 aa) |
| | TCTN2 | Tectonic family member 2. (665 aa) |
| | UNC119B | Unc-119 lipid binding chaperone B. (254 aa) |
| | BBS12 | BBS12 protein. (708 aa) |
| | BBS4 | Bardet-Biedl syndrome 4 protein homolog; May be required for the dynein-mediated transport of pericentriolar proteins to the centrosome. Required for microtubule anchoring at the centrosome but not for microtubule nucleation. The BBSome complex is required for ciliogenesis but is dispensable for centriolar satellite function. This ciliogenic function is mediated in part by the Rab8 GDP/GTP exchange factor, which localizes to the basal body and contacts the BBSome. Rab8(GTP) enters the primary cilium and promotes extension of the ciliary membrane. Firstly the BBSome associates with the [...] (519 aa) |
| | NPHP1 | Nephrocystin 1. (686 aa) |
| | IFT20 | Intraflagellar transport protein 20 homolog; Part of intraflagellar transport (IFT) particles involved in ciliary process assembly. May play a role in the trafficking of ciliary membrane proteins from the Golgi complex to the cilium. Regulates the platelet-derived growth factor receptor-alpha (PDGFRA) signaling pathway. Required for protein stability of E3 ubiquitin ligases CBL and CBLB that mediate ubiquitination and internalization of PDGFRA for proper feedback inhibition of PDGFRA signaling. Essential for male fertility. Plays an important role in spermatogenesis, particularly sperm [...] (156 aa) |
| | IHH | Hedgehog protein; Intercellular signal essential for a variety of patterning events during development. (412 aa) |
| | ARL6 | ADP-ribosylation factor-like protein 6; Involved in membrane protein trafficking at the base of the ciliary organelle. Mediates recruitment onto plasma membrane of the BBSome complex which would constitute a coat complex required for sorting of specific membrane proteins to the primary cilia. Together with the BBSome complex and LTZL1, controls SMO ciliary trafficking and contributes to the sonic hedgehog (SHH) pathway regulation. May regulate cilia assembly and disassembly and subsequent ciliary signaling events such as the Wnt signaling cascade. Isoform 2 may be required for proper r [...] (186 aa) |
| | BBS5 | Bardet-Biedl syndrome 5 protein homolog; The BBSome complex is thought to function as a coat complex required for sorting of specific membrane proteins to the primary cilia. The BBSome complex is required for ciliogenesis but is dispensable for centriolar satellite function. This ciliogenic function is mediated in part by the Rab8 GDP/GTP exchange factor, which localizes to the basal body and contacts the BBSome. Rab8(GTP) enters the primary cilium and promotes extension of the ciliary membrane. Firstly the BBSome associates with the ciliary membrane and binds to RAB3IP/Rabin8, the gua [...] (341 aa) |
| | GLI2 | GLI family zinc finger 2. (1431 aa) |
| | RCC1L | RCC1 like. (464 aa) |
| | TCTN3 | Tectonic family member 3. (599 aa) |
| | IFT43 | Intraflagellar transport protein 43 homolog; As a component of IFT complex A (IFT-A), a complex required for retrograde ciliary transport and entry into cilia of G protein- coupled receptors (GPCRs), it is involved in ciliogenesis. Involved in retrograde ciliary transport along microtubules from the ciliary tip to the base. (207 aa) |
| | IFT46 | Intraflagellar transport protein 46 homolog; Forms part of a complex involved in intraflagellar transport (IFT), the bi-directional movement of particles required for the assembly, maintenance and functioning of primary cilia. May play a role in chondrocyte maturation and skeletogenesis (By similarity). (301 aa) |
| | IQCB1 | IQ motif containing B1. (598 aa) |
| | UNC119 | Protein unc-119 homolog A; Involved in synaptic functions in photoreceptor cells, the signal transduction in immune cells as a Src family kinase activator, endosome recycling, the uptake of bacteria and endocytosis, protein trafficking in sensory neurons and as lipid-binding chaperone with specificity for a diverse subset of myristoylated proteins. Specifically binds the myristoyl moiety of a subset of N-terminally myristoylated proteins and is required for their localization. Binds myristoylated GNAT1 and is required for G-protein localization and trafficking in sensory neurons. Proba [...] (240 aa) |
| | SMO | Smoothened, frizzled class receptor; Belongs to the G-protein coupled receptor Fz/Smo family. (780 aa) |
| | TTC30B | Tetratricopeptide repeat protein 30B; Required for polyglutamylation of axonemal tubulin. Plays a role in anterograde intraflagellar transport (IFT), the process by which cilia precursors are transported from the base of the cilium to the site of their incorporation at the tip; Belongs to the TTC30/dfy-1/fleer family. (664 aa) |
| | BOC | BOC cell adhesion associated, oncogene regulated. (1115 aa) |
| | LOC112441540 | ANK_REP_REGION domain-containing protein. (362 aa) |
| | IQCE | IQ motif containing E. (721 aa) |
| | WDR19 | WD repeat domain 19. (1342 aa) |
| | CCDC28B | Coiled-coil domain containing 28B. (246 aa) |
| | NEK8 | NIMA related kinase 8. (692 aa) |
| | TULP3 | Tubby-like protein; Belongs to the TUB family. (478 aa) |
| | HHIP | Hedgehog interacting protein. (700 aa) |
| | NEK9 | NIMA related kinase 9. (977 aa) |
| | SDCCAG8 | Uncharacterized protein. (715 aa) |
| | IFT172 | Intraflagellar transport 172. (421 aa) |
| | CEP290 | Centrosomal protein of 290 kDa; Involved in early and late steps in cilia formation. Its association with CCP110 is required for inhibition of primary cilia formation by CCP110. May play a role in early ciliogenesis in the disappearance of centriolar satellites and in the transition of primary ciliar vesicles (PCVs) to capped ciliary vesicles (CCVs). Required for the centrosomal recruitment of RAB8A and for the targeting of centriole satellite proteins to centrosomes such as of PCM1. Required for the correct localization of ciliary and phototransduction proteins in retinal photorecepto [...] (1453 aa) |
| | NEK6 | NIMA related kinase 6; Belongs to the protein kinase superfamily. (313 aa) |
| | IFT74 | Intraflagellar transport 74. (600 aa) |
| | BBS1 | BBS1 domain-containing protein. (667 aa) |
| | LOC507423 | Olfactory receptor. (316 aa) |
| | SCUBE2 | Signal peptide, CUB domain and EGF like domain containing 2. (1027 aa) |
| | VWA1 | Von Willebrand factor A domain-containing protein 1; Promotes matrix assembly. (492 aa) |
| | TMEM216 | Transmembrane protein 216; Part of the tectonic-like complex which is required for tissue-specific ciliogenesis and may regulate ciliary membrane composition. (141 aa) |
| | CUL3 | Cullin 3; Belongs to the cullin family. (768 aa) |
| | B9D2 | B9 domain-containing protein 2; Component of the tectonic-like complex, a complex localized at the transition zone of primary cilia and acting as a barrier that prevents diffusion of transmembrane proteins between the cilia and plasma membranes. (175 aa) |
| | BBS2 | Bardet-Biedl syndrome 2 protein homolog. (721 aa) |
| | PTCH2 | Patched 2. (1156 aa) |
| | GLI3 | GLI family zinc finger 3. (1587 aa) |
| | TTLL10 | Tubulin tyrosine ligase like 10. (679 aa) |
| | SHH | Hedgehog protein; Intercellular signal essential for a variety of patterning events during development. (461 aa) |
| | IFT122 | Intraflagellar transport 122. (1182 aa) |
| | SCUBE1 | Signal peptide, CUB domain and EGF like domain containing 1. (1018 aa) |
| | IFT27 | Intraflagellar transport protein 27 homolog; Small GTPase-like component of the intraflagellar transport (IFT) complex B that promotes the exit of the BBSome complex from cilia via its interaction with ARL6. Not involved in entry of the BBSome complex into cilium. Prevents aggregation of GTP-free ARL6. Required for hedgehog signaling. Forms a subcomplex within the IFT complex B with IFT25. Its role in intraflagellar transport is mainly seen in tissues rich in ciliated cells such as kidney and testis. Essential for male fertility, spermiogenesis and sperm flagella formation. Plays a rol [...] (186 aa) |
| | STK36 | Serine/threonine kinase 36. (1315 aa) |
| | TTC30A | Tetratricopeptide repeat protein 30A; Required for polyglutamylation of axonemal tubulin. Plays a role in anterograde intraflagellar transport (IFT), the process by which cilia precursors are transported from the base of the cilium to the site of their incorporation at the tip; Belongs to the TTC30/dfy-1/fleer family. (664 aa) |
| | TTC8 | Tetratricopeptide repeat domain 8. (501 aa) |
| | TTC26 | Tetratricopeptide repeat domain 26. (554 aa) |
| | IFT80 | Intraflagellar transport 80. (760 aa) |
| | TMEM17 | Transmembrane protein 17; Transmembrane component of the tectonic-like complex, a complex localized at the transition zone of primary cilia and acting as a barrier that prevents diffusion of transmembrane proteins between the cilia and plasma membranes. Required for ciliogenesis and sonic hedgehog/SHH signaling (By similarity). (198 aa) |
| | PUSL1 | tRNA pseudouridine synthase. (291 aa) |
| | NPHP4 | Nephrocystin 4. (1430 aa) |
| | IFT140 | Intraflagellar transport 140. (1457 aa) |
| | TTC21A | Tetratricopeptide repeat domain 21A. (724 aa) |
| | TRAF3IP1 | TRAF3 interacting protein 1. (686 aa) |
| | RP2 | Protein XRP2; Acts as a GTPase-activating protein (GAP) for tubulin in concert with tubulin-specific chaperone C, but does not enhance tubulin heterodimerization. (350 aa) |
| | MKS1 | MKS transition zone complex subunit 1. (558 aa) |
| | LOC112446756 | Uncharacterized protein. (407 aa) |
| | C2CD6 | C2 calcium dependent domain containing 6. (1847 aa) |
| | ANKS1B | ANKS1B protein. (509 aa) |
| | TMEM67 | Uncharacterized protein. (996 aa) |
| | MYO9A | Myosin IXA; Belongs to the TRAFAC class myosin-kinesin ATPase superfamily. Myosin family. (2340 aa) |
| | GAS1 | Growth arrest specific 1. (389 aa) |
| | CC2D2A | Coiled-coil and C2 domain containing 2A. (1620 aa) |
| | G3N118_BOVIN | Uncharacterized protein. (186 aa) |
| | G5E6L5_BOVIN | Uncharacterized protein. (807 aa) |
| | PTCH1 | SSD domain-containing protein. (1453 aa) |
| | TCTN1 | Tectonic family member 1. (588 aa) |
| | BBIP1 | Uncharacterized protein. (69 aa) |
| | MANEAL | Mannosidase endo-alpha like. (455 aa) |
| | CEP104 | Centrosomal protein 104. (993 aa) |
| | IFT81 | Intraflagellar transport 81. (698 aa) |
| | IFT52 | Intraflagellar transport 52 homolog (Chlamydomonas). (440 aa) |
| | DZIP1 | DAZ interacting zinc finger protein 1. (911 aa) |
| | KIF7 | Kinesin family member 7; Belongs to the TRAFAC class myosin-kinesin ATPase superfamily. Kinesin family. (1368 aa) |
| | PDE6D | Retinal rod rhodopsin-sensitive cGMP 3',5'-cyclic phosphodiesterase subunit delta; Promotes the release of prenylated target proteins from cellular membranes. Modulates the activity of prenylated or palmitoylated Ras family members by regulating their subcellular location (By similarity). Required for normal ciliary targeting of farnesylated target proteins, such as INPP5E (By similarity). Modulates the subcellular location of target proteins by acting as a GTP specific dissociation inhibitor (GDI) (By similarity). Increases the affinity of ARL3 for GTP by several orders of magnitude. [...] (160 aa) |
| | TMEM237 | Transmembrane protein 237; Component of the transition zone in primary cilia. Required for ciliogenesis (By similarity); Belongs to the TMEM237 family. (434 aa) |
| | WDR35 | WD repeat-containing protein 35; As a component of the IFT complex A (IFT-A), a complex required for retrograde ciliary transport and entry into cilia of G protein-coupled receptors (GPCRs), it is involved in ciliogenesis and ciliary protein trafficking. (1238 aa) |
| | CLUAP1 | Clusterin associated protein 1. (457 aa) |
| | ENSBTAP00000060141 | Uncharacterized protein. (147 aa) |
| | DLG5 | Discs large MAGUK scaffold protein 5; Belongs to the MAGUK family. (1946 aa) |
| | KIAA0556 | KIAA0556. (1673 aa) |
| | RPGRIP1L | RPGRIP1 like. (1270 aa) |
| | SAMD11 | Sterile alpha motif domain containing 11. (682 aa) |
| | TMEM231 | Transmembrane protein 231; Transmembrane component of the tectonic-like complex, a complex localized at the transition zone of primary cilia and acting as a barrier that prevents diffusion of transmembrane proteins between the cilia and plasma membranes. Required for ciliogenesis and sonic hedgehog/SHH signaling (By similarity). (348 aa) |
| | IFT22 | Intraflagellar transport protein 22 homolog. (199 aa) |
| | TMTC4 | Transmembrane O-mannosyltransferase targeting cadherins 4. (828 aa) |
| | SPATA20 | Spermatogenesis-associated protein 20; May play a role in fertility regulation. (789 aa) |
| | IFT22-2 | Intraflagellar transport protein 22 homolog; Small GTPase-like component of the intraflagellar transport (IFT) complex B; Belongs to the small GTPase superfamily. Rab family. (217 aa) |
| | SPOPL | Speckle type BTB/POZ protein like. (413 aa) |
| | RPGR | Uncharacterized protein. (732 aa) |
| | CDON | Cell adhesion associated, oncogene regulated. (1247 aa) |
| | ENSBTAP00000066185 | Uncharacterized protein. (237 aa) |
| | KIF27 | Kinesin family member 27; Belongs to the TRAFAC class myosin-kinesin ATPase superfamily. Kinesin family. (1395 aa) |
| | ENSBTAP00000068773 | EGF-like domain-containing protein. (208 aa) |
| | B9D1 | B9 domain containing 1. (239 aa) |
| | SUFU | Suppressor of fused homolog; Negative regulator in the hedgehog signaling pathway. Down- regulates GLI1-mediated transactivation of target genes. Part of a corepressor complex that acts on DNA-bound GLI1. May also act by linking GLI1 to BTRC and thereby targeting GLI1 to degradation by the proteasome. Sequesters GLI1, GLI2 and GLI3 in the cytoplasm, this effect is overcome by binding of STK36 to both SUFU and a GLI protein. Negative regulator of beta-catenin signaling. Regulates the formation of either the repressor form (GLI3R) or the activator form (GLI3A) of the full-length form of [...] (485 aa) |
| | EFCAB7 | EF-hand calcium binding domain 7. (629 aa) |
| | GPR161 | G protein-coupled receptor 161; Key negative regulator of Shh signaling, which promotes the processing of GLI3 into GLI3R during neural tube development. Recruited by TULP3 and the IFT-A complex to primary cilia and acts as a regulator of the PKA-dependent basal repression machinery in Shh signaling by increasing cAMP levels, leading to promote the PKA-dependent processing of GLI3 into GLI3R and repress the Shh signaling. In presence of SHH, it is removed from primary cilia and is internalized into recycling endosomes, preventing its activity and allowing activation of the Shh signalin [...] (573 aa) |
| | PLEKHA8 | Pleckstrin homology domain-containing family A member 8; Cargo transport protein that is required for apical transport from the trans-Golgi network (TGN). Transports AQP2 from the trans- Golgi network (TGN) to sites of AQP2 phosphorylation. Mediates the non- vesicular transport of glucosylceramide (GlcCer) from the trans-Golgi network (TGN) to the plasma membrane and plays a pivotal role in the synthesis of complex glycosphingolipids. Binding of both phosphatidylinositol 4-phosphate (PIP) and ARF1 are essential for the GlcCer transfer ability. Also required for primary cilium formation [...] (520 aa) |
| | CBY1 | Protein chibby homolog 1; Inhibits the Wnt/Wingless pathway by binding to CTNNB1/beta- catenin and inhibiting beta-catenin-mediated transcriptional activation through competition with TCF/LEF transcription factors. Has also been shown to play a role in regulating the intracellular trafficking of polycystin-2/PKD2 and possibly of other intracellular proteins. Promotes adipocyte and cardiomyocyte differentiation. Belongs to the chibby family. (158 aa) |
| | DISP1 | SSD domain-containing protein. (1555 aa) |
| | NEK7 | NIMA related kinase 7; Belongs to the protein kinase superfamily. (325 aa) |
| | ENSBTAP00000072807 | Uncharacterized protein. (166 aa) |
| | SCUBE3 | Signal peptide, CUB domain and EGF like domain containing 3. (1009 aa) |
| | BBS10 | Bardet-Biedl syndrome 10. (721 aa) |
| | IFT88 | Intraflagellar transport 88. (850 aa) |
| | AHI1 | Abelson helper integration site 1. (1172 aa) |
| | INVS | Inversin. (1108 aa) |
