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1 etalloproteinase, hemicentin, and a fat-like protocadherin).
2 Here we describe recent studies of the Fat1 protocadherin.
3 hat harbours the av mutation encodes a novel protocadherin.
4 neurons exhibit signal for subsets of delta-protocadherins.
5 ly of "nonclassic cadherin" genes designated protocadherins.
6 poER2; refs 9-11 ), alpha3beta1 integrin and protocadherins.
7 including the large family of cadherins and protocadherins.
9 eletions implicated genes, including PCDH10 (protocadherin 10) and DIA1 (deleted in autism1, or c3orf
11 nked proteins important in brain development-protocadherin 11 Y-linked (PCDH11Y) and neuroligin 4 Y-l
13 rmed by a tetramer of two cadherin proteins: protocadherin 15 (PCDH15) and cadherin 23 (CDH23), which
14 ents that consist of cadherin 23 (CDH23) and protocadherin 15 (PCDH15) and connect the stereocilia of
15 iated with deafness and hair bundle defects, protocadherin 15 (PCDH15) and myosin VIIa (MYO7A), into
16 e a static composition of the tip link, with protocadherin 15 (PCDH15) at the lower and cadherin 23 (
21 hair cells show that cadherin 23 (CDH23) and protocadherin 15 (PCDH15) localize to the upper and lowe
22 mains of cadherin 23 (CDH23), which binds to protocadherin 15 (PCDH15) to form tip links of mechanose
23 c hearing loss and an associated mutation in protocadherin 15 (Pcdh15), the Ames waltzer mice have be
27 utations in genes coding for cadherin 23 and protocadherin 15 cause deafness in both mice and humans.
28 full-length proteins; HCN1 can interact with protocadherin 15 CD3 and F-actin-binding filamin A formi
29 interact with HCN2 forming a complex without protocadherin 15 CD3 but including F-actin-binding fasci
30 ding sites for HCN1, with binding of HCN1 to protocadherin 15 CD3 favored between 26.5 and 68 microm
31 t interaction of the organ of Corti HCN1 and protocadherin 15 CD3 is mediated by amino-terminal seque
33 calcium chelators, binding between HCN1 and protocadherin 15 CD3 was characterized by a K(D) = 2.39
35 nteraction between HCN1 and tip-link protein protocadherin 15 CD3, a protein-protein interaction for
36 id analysis to bind the carboxyl terminus of protocadherin 15 CD3, a tip link protein implicated in m
39 rom our study show that both mouse and human protocadherin 15 genes have complex genomic structures a
40 ween HCN1 and stereociliary tip-link protein protocadherin 15 has been described for a teleost vestib
41 results further strengthen the importance of protocadherin 15 in the morphogenesis and cohesion of st
44 e and nonsense mutations of PCDH15, encoding protocadherin 15, are known to cause deafness and retini
49 ction channels, is composed of two proteins, protocadherin-15 and cadherin-23, whose mutation causes
51 resent the X-ray crystal structures of human protocadherin-15 EC8-EC10 and mouse EC9-EC10, which show
56 The new structure provides a first view of protocadherin-15's non-canonical EC linkers and suggests
57 LHFPL5 and TMC1 were shown to interact with protocadherin-15, a component of the tip link, which app
58 rs in the eye, as an avian ortholog of human protocadherin-15, a product of the gene for the deaf/bli
59 proteins-myosin VIIa, harmonin, cadherin-23, protocadherin-15, sans-do not display retinal degenerati
60 ced F-actin content in rods, suggesting that protocadherin-15-containing links are essential for thei
62 to the comparable region in trout hair cell protocadherin 15a-like protein, as necessary for binding
65 d 32 056 controls) revealing brain expressed protocadherin 17 (PCDH17) as a susceptibility gene for m
67 in the fish brain, with protocadherin-19 and protocadherin-17 showing much wider and stronger express
68 ession of three cadherins, protocadherin-19, protocadherin-17, and cadherin-6 in adult zebrafish brai
72 In this study, we demonstrate that pcdh 19 (protocadherin 19) acts synergistically with n-cadherin (
75 s genetic studies revealed that mutations in protocadherin-19 (Pcdh19) lead to an epilepsy syndrome w
77 t expression pattern in the fish brain, with protocadherin-19 and protocadherin-17 showing much wider
79 r, abducens, and vagal motor nuclei) contain protocadherin-19 and/or protocadherin-17-expressing cell
81 We have identified the zebrafish homolog of protocadherin-19, a member of the cadherin superfamily,
82 esults provide the first functional data for protocadherin-19, demonstrating an essential role in ear
83 examine mRNA expression of three cadherins, protocadherin-19, protocadherin-17, and cadherin-6 in ad
84 herin 7, contactin 3, netrin G2, cadherin 6, protocadherin 20, retinoid-related orphan receptor beta,
85 on studies showed that PROM1 interacted with protocadherin 21 (PCDH21), a photoreceptor-specific cadh
87 d mouse breast and lung cancer cells express protocadherin 7 (PCDH7), which promotes the assembly of
89 ed including Secreted Frizzled, cadherin 13, protocadherin 7, CCAAT/enhancer-binding protein-beta and
91 stent with the structural characteristics of protocadherins, a large first exon encodes the extracell
92 on profiles are observed for all eight delta-protocadherins along the dorsoventral, mediolateral, and
93 show that conditional deletion of the mouse protocadherin alpha (Pcdhalpha) gene cluster in serotone
95 P-containing loss-of-function alleles in the protocadherin alpha, beta and gamma gene clusters (Pcdha
99 oteins PCDH-J and FAT-J were classified into protocadherin and FAT-like subfamilies, respectively, ba
101 Two of these, PCDH18 and PCDH19, are novel protocadherins and a third is the human orthologue of mo
104 termined the genomic structure of this novel protocadherin, and found a single-base deletion in exon
105 matrix metalloproteinase, CDH-3, a Fat-like protocadherin, and hemicentin, a fibulin family extracel
107 proteins that includes classical cadherins, protocadherins, and atypical cadherins (Fat, Dachsous, a
117 a subgroup of the cadherin superfamily, the protocadherin beta (Pcdhbeta) cluster, was up-regulated
118 We show that loss of the apically localized protocadherin Cad99C rescues the SG defects, suggesting
120 re transcriptionally regulated by HLH-2: the protocadherin cdh-3, and two genes encoding secreted ext
122 results are consistent with a model in which protocadherin cis-dimers engage in a head-to-tail intera
124 ation in copy number and sequence content of protocadherin cluster genes among vertebrate species cou
130 em duplications have generated two zebrafish protocadherin clusters comprised of at least 97 genes.
133 , a synergistic underexpression of the gamma-protocadherin complex, located at Chr5q31 is also shown.
138 ed that a proximal to distal gradient of the protocadherin Dachsous (Ds) acts as a cue for planar cel
141 nt studies in Drosophila melanogaster of the protocadherins Dachsous and Fat suggest that they act as
142 polarity proteins, and another utilizing the protocadherins Dachsous and Fat, and the atypical myosin
144 rprisingly, O-mannosylation of cadherins and protocadherins does not require POMT1 and/or POMT2 in co
145 s showed that each of the investigated delta-protocadherins exhibits a spatially restricted and tempo
154 we have examined the functions of the giant protocadherin FAT by generating a transgenic mouse lacki
155 recent studies taken together show that the protocadherin Fat can regulate Warts in two different wa
156 y (PCP) signaling mediated by the Drosophila protocadherin Fat depends on its ability to change the s
160 he spatial distribution of bonds between the protocadherins Fat (Ft) and Dachsous (Ds), which form he
161 show that signaling interactions between the protocadherins Fat and Dachsous, organized by the morpho
165 the first evidence for the requirement of a protocadherin for normal function of the mammalian inner
166 ed one of these genes, a member of the gamma-protocadherins for further study to determine the basis
172 cadherins in the mouse, i.e., the clustered protocadherins, functions in an analogous fashion to pro
174 enes, (Ring1 and YY1 binding protein (RYBP); protocadherin gamma subfamily C,3 (PCDHGC3); and signal
179 ion molecule family encoded by the 22-member protocadherin-gamma (Pcdh-gamma) gene cluster in its con
182 gamma2-GABA(A)R) specifically interacts with protocadherin-gammaC5 (Pcdh-gammaC5) in the rat brain.
183 aptic cell adhesion molecules encoded by the protocadherin gene cluster are hypothesized to provide a
184 e by complete sequencing and analysis of the protocadherin gene cluster of the Indonesian coelacanth,
185 he encoded ectodomains of each member of the protocadherin gene clusters are present in one large exo
189 t we examined in greater detail involved the protocadherin gene family clusters on chromosome 5 (PCDH
191 populations, suggesting that a reduction in protocadherin gene number is not obviously deleterious.
192 se chromosome 10 contains Pcdh15, encoding a protocadherin gene that is mutated in ames waltzer and c
193 gamma and PCDH-alpha suggests that dozens of protocadherins generated by Pcdh-alpha and Pcdh-gamma ge
194 he combinatorial expression of the clustered protocadherin genes (Pcdhalpha, -beta, and -gamma).
195 mic organization of several additional human protocadherin genes by using DNA sequence information in
196 We sequenced the promoters of all 13 alpha protocadherin genes in 96 European Americans and identif
198 ge ectodomain exons are a general feature of protocadherin genes we have investigated the genomic org
199 rin 23, but differs significantly from other protocadherin genes, such as Pcdhalpha, beta, or gamma.
205 Among genes implicated by the top DMRs were protocadherins, homeobox genes, MAPKs and ryanodine rece
207 ly that adhesive interactions mediated by mu-protocadherin induce signaling events that regulate bran
209 n adhesive function by local expression of a protocadherin is a novel mechanism for controlling cell
212 res for the EC1-EC3 domain regions from four protocadherin isoforms representing the alpha, beta, and
213 that encompasses >70 genes at the clustered protocadherin locus (hereafter referred to as cPcdh).
215 ndrome lacking harmonin exhibits microvillar protocadherin mislocalization and severe defects in brus
217 tion factor Cdx2 activates expression of the protocadherin Mucdhl, which interacts with beta-catenin
219 Here, we demonstrate the requirement for NF-protocadherin (NFPC) and its cytosolic partner TAF1/Set
220 The homophilic cell adhesion molecule NF-protocadherin (NFPC) is expressed in the mid-dorsal opti
223 65% amino acid identity to Xenopus paraxial protocadherin (PAPC) and 42-49% amino acid identity to P
227 We previously showed that Xenopus paraxial protocadherin (PAPC) mediates cell sorting and morphogen
228 g, and demonstrated that it enables paraxial protocadherin (PAPC) to promote tissue separation throug
232 odulating complexes (AMCs) with the paraxial protocadherin (PAPC; denoted as AMCP) and C-cadherin (de
233 pus and zebrafish have demonstrated that the protocadherin, papc, is expressed in an anterior-specifi
234 Here we show that the expression of delta-protocadherins partitions the zebrafish optic tectum int
239 hromatin looping in mammalian genomes, using protocadherin (Pcdh) and beta-globin as model genes.
241 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
248 recently demonstrated that initiation of cdh/protocadherin (pcdh) O-Man glycosylation is not dependen
251 stinct repertoires of alpha, beta, and gamma protocadherin (Pcdh) proteins, which function in neural
252 ingle nucleotide polymorphisms (SNPs) in the protocadherin (PCDH)-alpha, -beta and -gamma gene cluste
258 ic approach to investigate the role of gamma-protocadherins (Pcdh-gammas) in hypothalamic neuronal ci
259 h4, Cdh6, Cdh7, Cdh8, Cdh11) and eight delta-protocadherins (Pcdh1, Pcdh7, Pcdh8, Pcdh9, Pcdh10, Pcdh
260 on, we placed the human homolog of the mouse protocadherin Pcdh15 in the linkage interval defined by
261 d99C, the Drosophila ortholog of human Usher protocadherin PCDH15, is expressed in several embryonic
263 es are free to make junctions containing the protocadherin, PCDH21, with the inner segment plasma mem
264 tosis-selective cell surface localization of protocadherin PCDH7, a member of a family with anti-adhe
267 Cs, both processes are mediated by the gamma-protocadherins (Pcdhgs), a family of 22 recognition mole
273 Here we demonstrate roles for the clustered protocadherins (Pcdhs) in dendritic self-avoidance and s
275 ession of alpha-, beta-, and gamma-clustered protocadherins (Pcdhs) provides vertebrate neurons with
277 sively, much less is known about the related protocadherins (Pcdhs), which together make up the major
280 racellular and transmembrane domains of each protocadherin protein are encoded by an unusually large
285 ely 120 diverse membrane proteins, including protocadherins, ROBOs, netrin receptors, neuroligins, GP
288 study, we cloned eight members of the delta-protocadherin subfamily of cadherins (Pcdh1, Pcdh7, Pcdh
289 these genes was found to be a member of the protocadherin subfamily, based on amino acid sequence co
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
293 functions of O-Man glycans on cadherins and protocadherins, we used a combinatorial gene-editing str
295 composed of protocadherin-24 and mucin-like protocadherin, which target to microvillar tips and inte
296 adherin-18a (Pcdh18a) belongs to the delta 2-protocadherins, which constitute the largest subgroup wi
297 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
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