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1 egorize the effects of missense mutations in polycystins.
5 c kidney disease (PKD), genetic mutations in polycystin 1 and 2 lead to defective intracellular traff
8 ons in the PKD1 and PKD2 genes, encoding the polycystin 1 and polycystin 2 Ca(2+) ion channels, respe
9 Coexpression of a cytoplasmic fragment of polycystin 1 blocks the PC2-TAZ interaction and prevents
13 hydrate or when ciliary-associated proteins polycystin-1 (a mechanoreceptor), polycystin-2 (a Ca2+ c
14 eceptor potential polycystin (TRPP) channels polycystin-1 (PC-1) and polycystin-2 (PC-2) act as a mec
16 TORC1 negatively regulates the biogenesis of polycystin-1 (PC-1) and trafficking of the PC-1/2 comple
19 aled that the protein and mRNA expression of polycystin-1 (PC1) and polycystin-2 (PC2) were increased
20 The ADPKD proteins encoded by these genes, polycystin-1 (PC1) and polycystin-2 (PC2), form a plasma
22 gous mutations in PKD1 or PKD2, which encode polycystin-1 (PC1) and polycystin-2 (PC2), respectively,
23 tations of the PKD1 and PKD2 genes, encoding polycystin-1 (PC1) and polycystin-2 (PC2), respectively,
24 is caused by mutations in the genes encoding polycystin-1 (PC1) and polycystin-2 (PC2), which form an
27 ory cilia of Caenorhabditis elegans neurons, polycystin-1 (PC1) and transient receptor polycystin-2 c
33 ll subunits of the BBSome, the ADPKD protein polycystin-1 (PC1) interacts with BBS1, BBS4, BBS5 and B
40 ning disorder, commonly caused by defects in polycystin-1 (PC1) or polycystin-2 (PC2), in which tubul
41 ve copy of either PKD1 or PKD2, which encode polycystin-1 (PC1) or polycystin-2 (PC2), respectively.
42 tion of either PC2 or its regulatory protein polycystin-1 (PC1) results in autosomal dominant polycys
43 acid sequence, KVHPSST, in the C-terminus of polycystin-1 (PC1) that serves as a ciliary-targeting si
47 eoblast/osteocyte expression and function of polycystin-1 (PC1), a transmembrane protein that is a co
48 suggests that fibrocystin/polyductin (FPC), polycystin-1 (PC1), and polycystin-2 (PC2) are all local
49 sease (ADPKD), which results from defects in polycystin-1 (PC1), but the function of PC1 remains poor
66 ncident collecting duct-specific knockout of polycystin-1 and AC6 (also homozygous for the floxed ADC
67 roteomic analysis confirmed that cleavage of polycystin-1 and fibrocystin occurs in vivo, in manners
70 cystin research, with a focus on the role of polycystin-1 and polycystin-2 in primary cilia and the c
71 y-membrane translocation of the PKD proteins polycystin-1 and polycystin-2 is compromised in DZIP1L-m
72 ycystic kidney disease (ADPKD) gene products polycystin-1 and polycystin-2 localize to both cilia and
75 consistent with cleavage at the GPS site in polycystin-1 and the proprotein convertase site in fibro
80 define genetic and biochemical modulators of polycystin-1 function and provide a more complete defini
82 inhibition increases steady-state levels of polycystin-1 in cells lacking glucosidase IIbeta and tha
84 demonstrate, for the first time, a role for polycystin-1 in kidney injury and repair and indicate th
91 PKD1 or with abnormal ciliary expression of polycystin-1 or -2 were not responsive to fluid shear st
94 ll) or Tg737(orpk/orpk) mutation encoded for polycystin-1 or polaris, respectively, are unable to tra
99 8 interacted with polycystin-2, but not with polycystin-1, and that the jck mutation did not affect t
100 D, which displays a hypomorphic phenotype of polycystin-1, demonstrated increased cyst progression an
101 Vs) and isolated a subpopulation abundant in polycystin-1, fibrocystin (in their cleaved forms), and
102 e investigated the role of the intracellular polycystin-1, lipoxygenase, and alpha-toxin (PLAT) signa
103 gene encodes a large transmembrane protein (polycystin-1, or PC-1) that is reported to function as a
104 cyte cilia are sensory organelles containing polycystin-1, polycystin-2, and adenylyl cyclase isoform
105 tosomal recessive polycystic kidney disease (polycystin-1, polycystin-2, and fibrocystin) localize to
106 inergic Ca(2+) signaling may be regulated by polycystin-1, since ORPK cells only expressed the C-term
107 s in defective maturation and trafficking of polycystin-1, the central determinant of cyst pathogenes
109 regulated in cyst-lining epithelial cells in polycystin-1-deficient murine kidneys and accumulated in
113 yst development, either in genetic models of polycystin-1/2 reduction or in response to ischemia/repe
114 um binding from the membrane-targeting PLAT (polycystin-1/lipoxygenase/alpha-toxin) domain to the act
115 a role by which GPSM1 increased heteromeric polycystin-1/polycystin-2 ion channel activity via Gbeta
116 ase complex that targets the ciliary protein polycystin 2 (PC2) for degradation, but whether Nek and
118 The calcium-permeable cation channel protein polycystin 2 (PC2) is overexpressed in kidneys of TAZ-/-
119 nvestigate the effect of decreased levels of polycystin 2 (PC2), a calcium channel that interacts wit
120 c1 functions by modulating the expression of polycystin 2 (Pkd2), a member of the transient receptor
123 olycystin-1 and transient receptor potential polycystin 2 (TRPP2) account for almost all clinically i
125 iliary function through its association with polycystin 2 and provides evidence of a further link bet
126 tin 2, dual morpholino-mediated knockdown of polycystin 2 and RP2 results in enhanced situs inversus,
129 nd PKD2 genes, encoding the polycystin 1 and polycystin 2 Ca(2+) ion channels, respectively, result i
130 lization of the transient receptor potential polycystin 2 channel (TRPP2/PKD-2) is evolutionarily con
133 ented the dorsal axis curvature formation in polycystin 2 morphants and curly up polycystin 2 mutants
136 s canonical binding site (EF-hand domain) of polycystin 2, a Ca(2+)-dependent channel with relevance
137 bserved physical interaction between RP2 and polycystin 2, dual morpholino-mediated knockdown of poly
138 ased notochord sheath collagen deposition in polycystin 2-deficient embryos is directly linked to axi
142 d proteins polycystin-1 (a mechanoreceptor), polycystin-2 (a Ca2+ channel), and the Ca2+-inhibitable
143 stin (TRPP) channels polycystin-1 (PC-1) and polycystin-2 (PC-2) act as a mechanosensitive channel, w
146 in/polyductin (FPC), polycystin-1 (PC1), and polycystin-2 (PC2) are all localized at the plasma membr
150 at PC1 co-localizes with the calcium channel polycystin-2 (PC2) in primary cilia of MC3T3-E1 osteobla
156 ied a missense mutation in the gene encoding polycystin-2 (PC2) that prevented this protein from prop
157 nd mRNA expression of polycystin-1 (PC1) and polycystin-2 (PC2) were increased in jck mouse kidneys.
159 coded by these genes, polycystin-1 (PC1) and polycystin-2 (PC2), form a plasma membrane receptor-ion
160 y caused by defects in polycystin-1 (PC1) or polycystin-2 (PC2), in which tubular epithelia form flui
161 D proteins, termed as polycystin-1 (PC1) and polycystin-2 (PC2), interact via their C-termini to form
162 or PKD2, which encode polycystin-1 (PC1) and polycystin-2 (PC2), respectively, cause autosomal domina
163 PKD2 genes, encoding polycystin-1 (PC1) and polycystin-2 (PC2), respectively, lead to autosomal domi
165 sensation opens the calcium (Ca(2+)) channel polycystin-2 (PC2), resulting in a calcium flux-mediated
166 and co-localizes with the Pkd2 gene product polycystin-2 (PC2), suggesting that these two proteins m
169 in the genes encoding polycystin-1 (PC1) and polycystin-2 (PC2), which form an ion channel complex th
170 smembrane protein that is a component of the polycystin-2 (PC2)-ciliary mechano-sensor complex in ren
172 e genes encoding polycystin-1 (PC1, PKD1) or polycystin-2 (PC2, PKD2) cause ADPKD, and PKD1 mutations
177 us work has indicated that sensory cilia and polycystin-2 (Pkd2), a cation channel, are required for
180 biochemically and genetically interacts with polycystin-2 (the protein product of the polycystic kidn
182 pting the recycling endosome reduces ciliary polycystin-2 and causes its accumulation in the recyclin
184 Our analysis showed that fibrocystin and polycystin-2 are dependent on IFT20, GMAP210, and the ex
185 he activation of which may reduce functional polycystin-2 below a critical threshold, precipitating t
186 s, polycystin-1 (PC1) and transient receptor polycystin-2 channel (TRPP2 or PC2), function together a
187 changes in the activation of the associated polycystin-2 channel or other intracellular events media
189 Biophysical properties of lacrimal gland polycystin-2 channels were similar to those described fo
191 is of immunogold labeling revealed strongest polycystin-2 expression on the membranes of the endoplas
194 ls generated from ADPKD patients do not show polycystin-2 in the cilia and are unable to sense fluid
196 ich GPSM1 increased heteromeric polycystin-1/polycystin-2 ion channel activity via Gbetagamma subunit
197 ocation of the PKD proteins polycystin-1 and polycystin-2 is compromised in DZIP1L-mutant cells.
201 sease (ADPKD) gene products polycystin-1 and polycystin-2 localize to both cilia and EVs, act in the
202 n-2-expressing counterparts, suggesting that polycystin-2 may also play an important role in the regu
203 binding sites in the cytoplasmic tail caused Polycystin-2 mislocalization to the apical cell surface.
206 solated artery, we further show that ciliary polycystin-2 responds specifically to shear stress and n
209 Thus, aberrant expression or localization of polycystin-2 to cilia could promote high blood pressure
210 normal expression level and localization of polycystin-2 to cilia is required for the endothelial ci
211 ans with ADPKD, disrupts the localization of polycystin-2 to the plasma membrane and primary cilia th
212 s, whereas in the caudal pronephric segment, Polycystin-2 was concentrated in subapical cytoplasmic v
213 t specific; in the proximal nephron segment, Polycystin-2 was localized to basolateral cell membranes
214 ciliary localization of the membrane protein Polycystin-2, a protein playing an important role in the
215 sensory organelles containing polycystin-1, polycystin-2, and adenylyl cyclase isoform 6 through whi
216 ive polycystic kidney disease (polycystin-1, polycystin-2, and fibrocystin) localize to various subce
217 the transport of ciliary components such as polycystin-2, and partial loss of this enzyme is suffici
219 that PDE1A activity is altered downstream of polycystin-2, and suggest that PDE1A is a viable drug ta
220 kDa, pericentriolar material protein 1, and polycystin-2, as well as the Golgi distribution of its b
221 n of the cilium and the ciliary transport of polycystin-2, as well as to alter proliferation signals
223 ments demonstrated that Nek8 interacted with polycystin-2, but not with polycystin-1, and that the jc
224 l channel-forming isoforms of TRPP channels (polycystin-2, polycystin-L, and polycystin-2L2) were exp
227 ARL13B is required for the ciliary entry of polycystin-2, the protein mutated in autosomal dominant
228 ther exploration of the in vivo functions of Polycystin-2, this study examined its expression and fun
229 whose mutation impairs ciliary motility, and polycystin-2, whose ablation is associated with hydrocep
231 e induces cellular calcium release through a polycystin-2-dependent pathway, arrests Pkd1(-/-) cell g
232 owth factor-stimulated conditions than their polycystin-2-expressing counterparts, suggesting that po
236 PP channels (polycystin-2, polycystin-L, and polycystin-2L2) were expressed in adult mouse lacrimal g
237 y transduction, the mechanisms that regulate polycystin activity and localization, or ciliary membran
239 vel disease-associated protein families: The polycystins (ADPKD); fibrocystins (ARPKD); and meckelin.
241 al a pathway connecting TNF-alpha signaling, polycystins and cystogenesis, the activation of which ma
242 with the signal transduction pathways of the polycystins and may control the targeting of these cilia
243 les display normal ciliary expression of the polycystins and respond to fluid-flow shear stress with
245 ressed cyst growth following inactivation of polycystins and that the severity of cystic disease was
255 The overall function of the primary cilium-polycystin complex may be to sense and transduce environ
256 lar localization and channel activity of the polycystin complex through its interaction with the O2-s
257 geting mechanism, whereby Rabep1 couples the polycystin complex to a GGA1/Arl3-based ciliary traffick
262 Brefeldin A treatment after the onset of polycystin deficiency phenotypes reversed the curved axi
263 ators of cardiac hypertrophy, are targets of polycystin-dependent fluid stress sensing in renal epith
264 sh the existence of a new pathway defined by polycystin-dependent inhibition and cilia-dependent acti
266 low shear stress can be used as a readout of polycystin function and that loss of mechanosensation in
268 otein families, the most prominent being the polycystin gene family, whose products are found on the
269 IMA-related (never in mitosis A) kinase, and polycystins in jck cilia is shown for the first time.
271 Kidney cysts occur following inactivation of polycystins in otherwise intact cilia or following compl
272 These data suggest a novel role for the polycystins in sensing and responding to cellular O2 lev
274 disease 2-like 1 (Pkd2l1 or Pkdl), encoding polycystin-L (PCL), a non-selective cation channel, incr
275 ing isoforms of TRPP channels (polycystin-2, polycystin-L, and polycystin-2L2) were expressed in adul
281 logical experiments to determine whether the polycystins PC1 and PC2 (encoded by Pkd1 and Pkd2) and t
283 tations in Pkd1/Pkd2 and Pkhd1, which encode polycystins (PCs) and fibrocystin/polyductin (FPC).
285 localization of the kinesin-3 KLP-6 and the polycystin PKD-2 in male-specific sensory neurons in C.
289 ngth of time between the initial loss of the polycystin proteins and the subsequent involution of cil
292 d new pathways controlling EV biogenesis and polycystin signaling and also identified EV cargo, which
293 tivated protein kinase (MAPK) pmk-1 acted in polycystin-signaling pathways controlling male mating be
294 ipocyte differentiation, indicating that the polycystins/TAZ complex may be a potential therapeutic t
296 made of transient receptor potential channel polycystin (TRPP) and polycystic kidney disease (PKD) pr
297 dney cells, the transient receptor potential polycystin (TRPP) channels polycystin-1 (PC-1) and polyc
298 LOV-1 and PKD-2 transient receptor potential polycystin (TRPP) complex localizes to ciliated endings
299 se (ADPKD), a disease caused by mutations in polycystins, which are proteins that regulate signaling,
300 ses by combining conditional inactivation of polycystins with concomitant ablation of cilia in develo
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