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2 ed-1 (Ptch1) localizes to cilia and prevents ciliary accumulation and activation of smoothened (Smo).
5 reases in the expression of genes related to ciliary activity in vivo and alters multiple immune-rela
6 ed with greater epithelial damage with lower ciliary activity, cilium loss, less tight junction expre
8 rs in a characteristic hierarchy of bending, ciliary alteration, contractions, and detachment, which
9 material 1 in the mouse leads to progressive ciliary, anatomical, psychomotor, and cognitive abnormal
10 stnatal brain, with progressive degenerative ciliary and behavioral phenotypes; and they support a co
11 arises naturally as alternating sub-cellular ciliary and contractile activities rearrange the cell's
13 We propose that CFAP45 supports mammalian ciliary and flagellar beating via an adenine nucleotide
14 unction of the BBSome, a complex integral in ciliary and intracellular trafficking, in ribbon synapti
19 nd our understanding of the role of PACRG in ciliary assembly and motility, we used a combination of
22 ponents toward the ciliary base; second, the ciliary axoneme shortens and motors slow down; and third
23 S, we determined the structure of the intact ciliary axoneme with up to resolution of 12 angstrom.
25 expressed in ciliated tissues, localizes to ciliary axonemes and basal bodies, and is required for c
26 these are specifically expressed in the main ciliary band of the mollusc Tritia (also known as Ilyana
29 here we characterize the organization of the ciliary basal foot, an appendage of basal bodies whose m
32 mutant cells, mutant PC2 accumulates at the ciliary base, forming a ring pattern consistent with dis
36 ds, redistributing IFT components toward the ciliary base; second, the ciliary axoneme shortens and m
37 ized approaches for systematically assessing ciliary beat dynamics and for drug testing would improve
40 atter via an increase in LRRC26 expression), ciliary beat frequency and airway surface liquid volume
42 of the cilium, could perceive forces during ciliary beating and transfer these signals to locally re
44 autosomal recessive inheritance) that impair ciliary beating cause a variety of motile ciliopathies,
48 ral and third ventricles and deceleration of ciliary beating on ependymal cells lining the ventricula
49 ture is well-suited for mechanoregulation of ciliary beating through physical collisions with the CP.
55 onclude that FAM149B1 is required for normal ciliary biology and that its deficiency results in a ran
56 gment epithelium and photoreceptors, and the ciliary body and choroid plexus, the sources of aqueous
58 les that originate from the vasculature-rich ciliary body and extend along the surface of the lens ca
59 in the lens immune response and suggest the ciliary body as a source of immune cells to the avascula
60 aled a partially cystic mass adjacent to the ciliary body between the 5 and 9 o'clock meridians as we
62 neovascular glaucoma, and dose to 20% of the ciliary body had the largest impact on ocular hypertensi
63 the use of targeted chemotherapy to treat a ciliary body medulloepithelioma with aqueous and vitreou
65 me complete regression of a non-infiltrating ciliary body medulloepithelioma with seeding achieved wi
66 age points including the aortic root and the ciliary body of the eye and that systemic overexpression
69 quadrants of the angle: anteriorly directed ciliary body, absent ciliary sulcus, iris angulation, fl
70 and site (retina, C69.2; choroid, C69.3; and ciliary body, C69.4) from 1975 through 2011 using the Su
73 nonpigmented and pigmented epithelia of the ciliary body, lens epithelium, and a subset of retinal n
75 se 1 like 1 (PKD1L1), a gene associated with ciliary calcium signaling and embryonic laterality deter
76 negatively regulated by constitutively high ciliary cAMP maintained by the Galpha(s)-coupled GPCR, G
78 tigrella crozieri, and found it expressed in ciliary cells of eyes in the larva, and in extraocular c
79 microvillar cells with rhabdomeric opsin and ciliary cells with ciliary opsin, each type having speci
81 mes from the distal appendages (DAPs) to the ciliary compartment (CC), moving slowly in the DAPs, rel
82 c and monogenic mouse models highlighted the ciliary compartment as a common dysregulated target, wit
83 within the cytoplasm rather than within the ciliary compartment, are proposed to allow for the effic
85 g, as well as for the assembly of additional ciliary components that play essential roles in generati
87 reassessment of the relationship between the ciliary cytoskeleton and microtubule-based transport in
90 yos and mammalian cell culture, arguing that ciliary defects are at least partially involved in the p
93 ss indicate that two processes contribute to ciliary disassembly in response to cessation of anterogr
94 res of the major mechanoregulators that bind ciliary doublet microtubules in Chlamydomonas reinhardti
97 sor tooth epithelia as a model, we show that ciliary dynamics in stem cells require the proper functi
100 thies, a group of genetic diseases linked to ciliary dysfunction, and gene therapy is an attractive t
101 specified set of 2,016 genes associated with ciliary dysgenesis and/or dysfunction or cholestasis wer
105 otypes, some of which are typical of primary ciliary dyskinesia (PCD), a condition caused by motile c
106 nce, for example in cystic fibrosis, primary ciliary dyskinesia (PCD), and select immunodeficiencies(
107 atients with ciliopathies, including primary ciliary dyskinesia and Bardet-Biedl syndrome, also suffe
108 phic mutations of human DNAAF2 can result in ciliary dyskinesia and identify Dnaaf2 as an essential c
109 ause loss of cilia motility in human primary ciliary dyskinesia patients is not fully associated with
110 ired, the disorder is referred to as primary ciliary dyskinesia, the most common motile ciliopathy.
112 e is no evidence-based treatment for primary ciliary dyskinesia; therapies aim at relieving symptoms
113 sence of PAM in mammalian cilia suggest that ciliary ectosome-mediated peptidergic signaling dates to
114 rtases, amidated products, and receptors) in ciliary ectosomes from the green alga Chlamydomonas.
115 iffusion-binding-transport model showed that ciliary enrichment of a rhodopsin kinase probe occurs vi
116 eletion of residues 261-275 of IFT54 reduced ciliary entry and anterograde traffic of IFT dynein with
117 ther with Thm2, regulates cilia disassembly, ciliary entry of membrane-associated protein, Hedgehog s
121 c analysis and open-flap biopsy specimens of ciliary epithelial and iris epithelial neoplasms, melano
122 to the variable cytokeratin staining in the ciliary epithelial neoplasms and the variable Lin28A sta
125 a common dysregulated target, with enhanced ciliary expression and length changes in the digenic mod
129 expression of downstream genes that modulate ciliary function and inflammatory response to influence
133 rgetable signaling axis that controls motile ciliary function in humans and has potential implication
136 s for PKD1L1 in laterality determination and ciliary function suggest that PKD1L1 is a biologically p
141 ly regulates the functional component of the ciliary gate and suggest a correlation between defective
145 pathies, the mechanism by which mutations in ciliary genes lead to bile duct developmental abnormalit
149 R GPR161 demonstrates that ubiquitination of ciliary GPCRs is required for their regulated exit from
150 re required for the signal-dependent exit of ciliary GPCRs, but the functional interplay between beta
151 ould potentially serve as one of the crucial ciliary growth checkpoints and could be channeled to dev
159 lates the motile ciliary proteome to promote ciliary length and mucociliary transport but which is di
160 ilia tip, was identified to be essential for ciliary length control through its microtubule depolymer
163 of V2R, however, does not have any effect on ciliary length, although the effect of tolvaptan on cili
167 se data provide a mechanism to control Smo's ciliary level during Hedgehog signaling by regulating th
171 gether, these results suggest that mammalian ciliary loss involves a tunable decision between decilia
172 of WDR92 expression in planaria resulted in ciliary loss, reduced beat frequency and dyskinetic moti
174 with a progenitor population residing in the ciliary marginal zone (CMZ) at the retinal peripheral ma
176 tants also possessed a reduced proliferative ciliary marginal zone, and decreased and disorganized Mu
177 gametes rapidly becomes enriched in the peri-ciliary membrane and enters the cilia to become active a
179 o regulate the actin-driven expansion of the ciliary membrane at the initiation of new outer segment
180 that the organization of cholesterol in the ciliary membrane can be modified by extracellular ligand
181 3), which controls sequential recruitment of ciliary membrane components, histone deacetylase, and tr
183 naling pathway that links nuclear enzymes to ciliary membrane receptors via TULP3, describes a dynami
187 geting of lipidated Rab28 to periciliary and ciliary membranes is highly dependent on the BBSome and
192 rd1 mechanism contributes to deceleration of ciliary motility and age-dependent ventricular enlargeme
200 concept uses the (remaining) strength of the ciliary muscle, whereas another is triggered by the pupi
201 om neighboring tissues, predominantly in the ciliary muscle/scleral spur, which together correspond t
203 e family, including oncostatin (OSM), IL-11, ciliary neurotrophic factor (CNTF), and leukemia inhibit
204 phic factor, fibroblast growth factor-2, and ciliary neurotrophic factor (CNTF), and the implantation
205 E3 inhibits OPC proliferation by stimulating ciliary neurotrophic factor (CNTF)-mediated signaling th
206 cardiotrophin-like cytokine factor 1 (CLCF1)-ciliary neurotrophic factor receptor (CNTFR) signaling a
208 ransport but which is dispensable for normal ciliary number, radial structure, and beat frequency.
209 ith rhabdomeric opsin and ciliary cells with ciliary opsin, each type having specialized molecular co
210 rst organism known to have both xenopsin and ciliary opsin, showing that these opsins are not necessa
211 mics of the probes, including presynapse and ciliary OS enrichment, depending on lipid moiety and pro
215 hat EXOC5 mRNA almost completely rescues the ciliary phenotypes in exoc5-mutant zebrafish, unlike the
217 ssential for Shh signal transduction and the ciliary phosphatidylinositol phosphatase Inpp5e is linke
219 Ns and EHDs are dynamically localized to the ciliary pocket membrane (CPM) and transported away from
222 s that this mechanism evolved from an innate ciliary process of releasing small extracellular vesicle
225 hat targeted exon skipping is able to rescue ciliary protein composition defects, provides functional
226 novel insights into the relationship between ciliary protein dysfunction and neuropsychiatric disorde
227 e emerging roles for RanGTP/importin beta in ciliary protein targeting, we further investigated the e
233 iodigital syndrome 1 (Ofd1), which encodes a ciliary protein, in Wnt1-Cre; Ofdfl/Y mice led to a comp
234 les of intraflagellar transport (IFT), where ciliary proteins are transported between the ciliary bas
235 that Ub chains mark GPCRs and other unwanted ciliary proteins for recognition by the ciliary exit mac
236 a deeper systems-level understanding of how ciliary proteins function together may provide new mecha
239 wever, the mechanisms by which PC1 and other ciliary proteins traffic to the primary cilium remain po
240 that mutations in genes coding for multiple ciliary proteins underlie polycystic kidney disease, a d
242 esulting cilia "interactome" consists of 165 ciliary proteins, 1,011 known PPIs, and 765 novel PPIs.
243 nteractome revealed interconnections between ciliary proteins, and their relation to several pathways
244 c kinase whose activity regulates the motile ciliary proteome to promote ciliary length and mucocilia
245 ary targeting sequence (CTS) that resides in ciliary receptors is considered to function in the proce
246 d disorders manifested by mislocalization of ciliary receptors.SIGNIFICANCE STATEMENT Retinal ciliopa
249 ryonic fibroblasts (MEF), we investigate the ciliary roles of two mammalian orthologues of Chlamydomo
251 that trigger cilia formation, extension and ciliary signal transduction are still not fully understo
254 that promoted the asymmetric inheritance of ciliary signaling components and supported cilium reasse
256 tbk2 knockout, indicating that disruption of ciliary signaling is a key driver of these phenotypes.
259 s (PI3K, AKT, and AC3) commonly regulated by ciliary signaling receptors induce rapid changes in axon
261 strate how defects in the balanced output of ciliary signalling events are coupled to developmental d
262 og (HH) pathway, for example, is a bona fide ciliary signalling system that regulates cell fate and s
263 sease, such as dysregulated mTOR and primary ciliary signalling, have roles in metabolic regulation a
266 ology of Bbs2-/- mice by directly preserving ciliary structure and function towards a normal phenotyp
267 tudies have identified mutations that affect ciliary structure and nucleation in PCD(4), but the regu
268 ese effects are secondary to preservation of ciliary structure and signaling, and stimulation of cell
271 evice outside the eye was implanted into the ciliary sulcus with or without trans-scleral suture fixa
272 le: anteriorly directed ciliary body, absent ciliary sulcus, iris angulation, flat iris plane, and ir
277 in phosphatase 1alpha interacts with a novel ciliary targeting sequence of polycystin-1 and regulates
281 We show that conditional knockout of the key ciliary trafficking gene Ift88 in adult mice results in
283 otential function of SMO in regulating basal ciliary trafficking of GLI2 when the pathway is off.
284 e that dictates nuclear transport, regulates ciliary trafficking of KAP3, a key component for functio
285 at the tubby family protein Tulp3 determines ciliary trafficking of polycystins in kidney collecting
287 m to activate distinct sets of target genes (ciliary transcription factor genes versus basal body amp
289 ion of glycolysis in mammalian cells induces ciliary translocation of Smo-a key step in pathway activ
296 rved in the structural maps, supporting that ciliary vesicles budding may serve as ectosomes for cell
298 ese results demonstrate that lens-associated ciliary zonules are directly involved in the lens immune
299 icrofibril-associated protein-1 (MAGP1)-rich ciliary zonules that originate from the vasculature-rich