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1 etalloproteinase, hemicentin, and a fat-like protocadherin).
2 Here we describe recent studies of the Fat1 protocadherin.
3 ibosome function, DNA binding, and clustered protocadherins.
4 including the large family of cadherins and protocadherins.
5 neurons exhibit signal for subsets of delta-protocadherins.
7 eletions implicated genes, including PCDH10 (protocadherin 10) and DIA1 (deleted in autism1, or c3orf
9 nked proteins important in brain development-protocadherin 11 Y-linked (PCDH11Y) and neuroligin 4 Y-l
10 rmed by a tetramer of two cadherin proteins: protocadherin 15 (PCDH15) and cadherin 23 (CDH23), which
11 entous tip links, consisting of the proteins protocadherin 15 (PCDH15) and cadherin 23 (CDH23), whose
12 ents that consist of cadherin 23 (CDH23) and protocadherin 15 (PCDH15) and connect the stereocilia of
13 iated with deafness and hair bundle defects, protocadherin 15 (PCDH15) and myosin VIIa (MYO7A), into
14 e a static composition of the tip link, with protocadherin 15 (PCDH15) at the lower and cadherin 23 (
18 ch of these links comprises two molecules of protocadherin 15 (PCDH15) joined to two of cadherin 23 (
19 hair cells show that cadherin 23 (CDH23) and protocadherin 15 (PCDH15) localize to the upper and lowe
20 mains of cadherin 23 (CDH23), which binds to protocadherin 15 (PCDH15) to form tip links of mechanose
23 utations in genes coding for cadherin 23 and protocadherin 15 cause deafness in both mice and humans.
24 full-length proteins; HCN1 can interact with protocadherin 15 CD3 and F-actin-binding filamin A formi
25 interact with HCN2 forming a complex without protocadherin 15 CD3 but including F-actin-binding fasci
26 ding sites for HCN1, with binding of HCN1 to protocadherin 15 CD3 favored between 26.5 and 68 microm
27 t interaction of the organ of Corti HCN1 and protocadherin 15 CD3 is mediated by amino-terminal seque
29 calcium chelators, binding between HCN1 and protocadherin 15 CD3 was characterized by a K(D) = 2.39
31 nteraction between HCN1 and tip-link protein protocadherin 15 CD3, a protein-protein interaction for
32 id analysis to bind the carboxyl terminus of protocadherin 15 CD3, a tip link protein implicated in m
34 of an heterotypic complex of cadherin 23 and protocadherin 15 ectodomain dimers, connect each stereoc
36 rom our study show that both mouse and human protocadherin 15 genes have complex genomic structures a
37 ween HCN1 and stereociliary tip-link protein protocadherin 15 has been described for a teleost vestib
39 ther components of the MT complex, including protocadherin 15, cadherin 23, lipoma HMGIC fusion partn
45 e we report the X-ray crystal structure of a protocadherin-15 + cadherin-23 heterotetrameric complex
47 ction channels, is composed of two proteins, protocadherin-15 and cadherin-23, whose mutation causes
49 esolution, depicting a parallel homodimer of protocadherin-15 EC1-3 molecules forming an antiparallel
50 resent the X-ray crystal structures of human protocadherin-15 EC8-EC10 and mouse EC9-EC10, which show
51 which a structurally diverse and multimodal protocadherin-15 ectodomain can act as a stiff or soft g
54 In addition, we report structures for 10 protocadherin-15 fragments used to build complete high-r
56 ropose models for the complete extracellular protocadherin-15 parallel homodimer and the tip-link bon
59 The new structure provides a first view of protocadherin-15's non-canonical EC linkers and suggests
60 d inner-ear sensory perception and elucidate protocadherin-15's structural and adhesive properties re
61 LHFPL5 and TMC1 were shown to interact with protocadherin-15, a component of the tip link, which app
62 rs in the eye, as an avian ortholog of human protocadherin-15, a product of the gene for the deaf/bli
63 proteins-myosin VIIa, harmonin, cadherin-23, protocadherin-15, sans-do not display retinal degenerati
64 ced F-actin content in rods, suggesting that protocadherin-15-containing links are essential for thei
66 d proper trafficking of known MET components Protocadherin 15a (Pcdh15a), Lipoma HMGIC fusion partner
67 to the comparable region in trout hair cell protocadherin 15a-like protein, as necessary for binding
70 d 32 056 controls) revealing brain expressed protocadherin 17 (PCDH17) as a susceptibility gene for m
72 in the fish brain, with protocadherin-19 and protocadherin-17 showing much wider and stronger express
73 ession of three cadherins, protocadherin-19, protocadherin-17, and cadherin-6 in adult zebrafish brai
77 y, TBR2 directly regulates the expression of Protocadherin 19 (PCDH19), and simultaneous PCDH19 expre
78 In this study, we demonstrate that pcdh 19 (protocadherin 19) acts synergistically with n-cadherin (
79 s of cadherins (N-cadherin, cadherin 11, and protocadherin 19) results in homotypic preference ex viv
82 s genetic studies revealed that mutations in protocadherin-19 (Pcdh19) lead to an epilepsy syndrome w
84 t expression pattern in the fish brain, with protocadherin-19 and protocadherin-17 showing much wider
86 r, abducens, and vagal motor nuclei) contain protocadherin-19 and/or protocadherin-17-expressing cell
88 We have identified the zebrafish homolog of protocadherin-19, a member of the cadherin superfamily,
89 esults provide the first functional data for protocadherin-19, demonstrating an essential role in ear
90 examine mRNA expression of three cadherins, protocadherin-19, protocadherin-17, and cadherin-6 in ad
91 herin 7, contactin 3, netrin G2, cadherin 6, protocadherin 20, retinoid-related orphan receptor beta,
92 on studies showed that PROM1 interacted with protocadherin 21 (PCDH21), a photoreceptor-specific cadh
94 ied GluN1-NTD protein as a bait, we identify Protocadherin 7 (PCDH7) as a potential interacting prote
95 d mouse breast and lung cancer cells express protocadherin 7 (PCDH7), which promotes the assembly of
99 on profiles are observed for all eight delta-protocadherins along the dorsoventral, mediolateral, and
100 show that conditional deletion of the mouse protocadherin alpha (Pcdhalpha) gene cluster in serotone
103 logy downstream of lowered expression of key Protocadherin alpha genes as compared to healthy control
105 P-containing loss-of-function alleles in the protocadherin alpha, beta and gamma gene clusters (Pcdha
106 as gene promoters, particularly those of the protocadherin alpha, beta, and gamma gene families.
109 gulation of the 5-HT axon arborization gene, Protocadherin-alphac2, during postnatal development of f
110 e 3D single particle structures of clustered protocadherin, an elongated particle with previously elu
111 oteins PCDH-J and FAT-J were classified into protocadherin and FAT-like subfamilies, respectively, ba
114 matrix metalloproteinase, CDH-3, a Fat-like protocadherin, and hemicentin, a fibulin family extracel
115 proteins that includes classical cadherins, protocadherins, and atypical cadherins (Fat, Dachsous, a
120 d sensory epithelial cells employ homologous protocadherin-based adhesion complexes to remodel their
122 intermicrovillar adhesion complex (IMAC), a protocadherin-based complex found at the tips of brush b
123 a subgroup of the cadherin superfamily, the protocadherin beta (Pcdhbeta) cluster, was up-regulated
124 We show that loss of the apically localized protocadherin Cad99C rescues the SG defects, suggesting
126 re transcriptionally regulated by HLH-2: the protocadherin cdh-3, and two genes encoding secreted ext
129 results are consistent with a model in which protocadherin cis-dimers engage in a head-to-tail intera
131 ation in copy number and sequence content of protocadherin cluster genes among vertebrate species cou
137 em duplications have generated two zebrafish protocadherin clusters comprised of at least 97 genes.
140 , a synergistic underexpression of the gamma-protocadherin complex, located at Chr5q31 is also shown.
145 ere, we report that members of the clustered Protocadherins (cPCDHs) control GABAergic interneuron su
146 ed that a proximal to distal gradient of the protocadherin Dachsous (Ds) acts as a cue for planar cel
149 nt studies in Drosophila melanogaster of the protocadherins Dachsous and Fat suggest that they act as
150 polarity proteins, and another utilizing the protocadherins Dachsous and Fat, and the atypical myosin
152 rprisingly, O-mannosylation of cadherins and protocadherins does not require POMT1 and/or POMT2 in co
153 olation(15,17-20), structures of full-length protocadherin ectodomains have not been determined, and
154 lecular arrangement of full-length clustered protocadherin ectodomains in single-isoform self-recogni
155 s showed that each of the investigated delta-protocadherins exhibits a spatially restricted and tempo
158 odel of interaction energy for the clustered protocadherin family that measures the contributions of
164 recent studies taken together show that the protocadherin Fat can regulate Warts in two different wa
165 y (PCP) signaling mediated by the Drosophila protocadherin Fat depends on its ability to change the s
169 he spatial distribution of bonds between the protocadherins Fat (Ft) and Dachsous (Ds), which form he
170 show that signaling interactions between the protocadherins Fat and Dachsous, organized by the morpho
175 ed one of these genes, a member of the gamma-protocadherins for further study to determine the basis
182 cadherins in the mouse, i.e., the clustered protocadherins, functions in an analogous fashion to pro
184 enes, (Ring1 and YY1 binding protein (RYBP); protocadherin gamma subfamily C,3 (PCDHGC3); and signal
189 ion molecule family encoded by the 22-member protocadherin-gamma (Pcdh-gamma) gene cluster in its con
191 rmine the crystal structure of the clustered protocadherin gammaB4 ectodomain, which reveals a zipper
192 -electron tomography, we show that clustered protocadherin gammaB6 ectodomains tethered to liposomes
193 gamma2-GABA(A)R) specifically interacts with protocadherin-gammaC5 (Pcdh-gammaC5) in the rat brain.
194 aptic cell adhesion molecules encoded by the protocadherin gene cluster are hypothesized to provide a
195 e by complete sequencing and analysis of the protocadherin gene cluster of the Indonesian coelacanth,
199 t we examined in greater detail involved the protocadherin gene family clusters on chromosome 5 (PCDH
200 gamma and PCDH-alpha suggests that dozens of protocadherins generated by Pcdh-alpha and Pcdh-gamma ge
201 he combinatorial expression of the clustered protocadherin genes (Pcdhalpha, -beta, and -gamma).
202 rin 23, but differs significantly from other protocadherin genes, such as Pcdhalpha, beta, or gamma.
203 ophrenia cINs had dysregulated expression of protocadherin genes, which lie within documented schizop
208 Among genes implicated by the top DMRs were protocadherins, homeobox genes, MAPKs and ryanodine rece
209 of sCAMs, including neurexins, neuroligins, protocadherins, immunoglobulin superfamily proteins, and
212 n adhesive function by local expression of a protocadherin is a novel mechanism for controlling cell
214 res for the EC1-EC3 domain regions from four protocadherin isoforms representing the alpha, beta, and
215 mammalian genome has more than 50 clustered protocadherin isoforms, which have remarkable homophilic
216 that encompasses >70 genes at the clustered protocadherin locus (hereafter referred to as cPcdh).
217 isoform-mismatch chain-termination model of protocadherin-mediated self-recognition, which depends o
218 ndrome lacking harmonin exhibits microvillar protocadherin mislocalization and severe defects in brus
220 tion factor Cdx2 activates expression of the protocadherin Mucdhl, which interacts with beta-catenin
222 Here, we demonstrate the requirement for NF-protocadherin (NFPC) and its cytosolic partner TAF1/Set
223 The homophilic cell adhesion molecule NF-protocadherin (NFPC) is expressed in the mid-dorsal opti
225 65% amino acid identity to Xenopus paraxial protocadherin (PAPC) and 42-49% amino acid identity to P
228 We previously showed that Xenopus paraxial protocadherin (PAPC) mediates cell sorting and morphogen
229 g, and demonstrated that it enables paraxial protocadherin (PAPC) to promote tissue separation throug
233 odulating complexes (AMCs) with the paraxial protocadherin (PAPC; denoted as AMCP) and C-cadherin (de
234 pus and zebrafish have demonstrated that the protocadherin, papc, is expressed in an anterior-specifi
235 Here we show that the expression of delta-protocadherins partitions the zebrafish optic tectum int
240 hromatin looping in mammalian genomes, using protocadherin (Pcdh) and beta-globin as model genes.
242 nd that Celsr3, a member of the nonclustered protocadherin (Pcdh) family, is predominantly expressed
246 f) binding sites, for example, the clustered protocadherin (Pcdh) genes, where we show Smchd1 and Ctc
247 recently demonstrated that initiation of cdh/protocadherin (pcdh) O-Man glycosylation is not dependen
249 stinct repertoires of alpha, beta, and gamma protocadherin (Pcdh) proteins, which function in neural
250 ingle nucleotide polymorphisms (SNPs) in the protocadherin (PCDH)-alpha, -beta and -gamma gene cluste
254 d that the combinatorial expression of gamma-protocadherins (Pcdh-gammas) and other clustered protoca
257 ic approach to investigate the role of gamma-protocadherins (Pcdh-gammas) in hypothalamic neuronal ci
258 h4, Cdh6, Cdh7, Cdh8, Cdh11) and eight delta-protocadherins (Pcdh1, Pcdh7, Pcdh8, Pcdh9, Pcdh10, Pcdh
259 d99C, the Drosophila ortholog of human Usher protocadherin PCDH15, is expressed in several embryonic
261 es are free to make junctions containing the protocadherin, PCDH21, with the inner segment plasma mem
262 tosis-selective cell surface localization of protocadherin PCDH7, a member of a family with anti-adhe
265 Here, we show that loss of clustered gamma protocadherins (Pcdhg), but not of genes in the alpha or
266 etion of the gene cluster encoding the gamma-Protocadherins (Pcdhgs) from developing GABAergic neuron
267 Cs, both processes are mediated by the gamma-protocadherins (Pcdhgs), a family of 22 recognition mole
268 he cadherin superfamily, the clustered gamma-Protocadherins (PCDHGs), regulate the survival of inhibi
273 Here we demonstrate roles for the clustered protocadherins (Pcdhs) in dendritic self-avoidance and s
275 ocadherins (Pcdh-gammas) and other clustered protocadherins (Pcdhs) provides a code of molecular iden
276 ession of alpha-, beta-, and gamma-clustered protocadherins (Pcdhs) provides vertebrate neurons with
278 sively, much less is known about the related protocadherins (Pcdhs), which together make up the major
284 erved between neurons that express identical protocadherin repertoires(2,5), and single-isoform diffe
285 on of ordered linear assemblies by clustered protocadherins represents the initial self-recognition s
287 ely 120 diverse membrane proteins, including protocadherins, ROBOs, netrin receptors, neuroligins, GP
289 study, we cloned eight members of the delta-protocadherin subfamily of cadherins (Pcdh1, Pcdh7, Pcdh
290 spatiotemporal expression patterns of delta-protocadherins suggest that they have multiple and diver
291 izing gene conversion events than coelacanth protocadherins, suggesting that recombination- and dupli
292 soforms of alpha-, beta- and gamma-clustered protocadherin that provide mammalian neurons with single
294 functions of O-Man glycans on cadherins and protocadherins, we used a combinatorial gene-editing str
296 composed of protocadherin-24 and mucin-like protocadherin, which target to microvillar tips and inte
297 adherin-18a (Pcdh18a) belongs to the delta 2-protocadherins, which constitute the largest subgroup wi
298 to be uniquely enlarged in vertebrates: the protocadherins, which regulate neuronal development, and
299 We propose a model in which association of a protocadherin with Ncad acts as a switch, converting bet