ライフサイエンスコーパス: peripherin

1 holine, calcitonin gene-related peptide, and peripherin).

2 ly labeled to detect alpha9, calretinin, and peripherin.

3 on endings, are not labeled by calretinin or peripherin.

4 t contain the alpha9 nAChR but did stain for peripherin.

5 in NOD mice recognizing the neuronal antigen peripherin.

6 tein (GFAP); and the nerve structural marker peripherin.

7 nied by gain of the tetraspanin rim protein, peripherin.

8 the absence of the outer segment protein rds/peripherin.

9 2 protein is 54%, identical to mammalian rds/peripherin.

10 , and a reduction in the level of normal rds/peripherin.

11 ucine (P216L) amino acid substitution in rds/peripherin.

12 TrkC, and RET and the sensory neurofilament peripherin.

13 photoreceptor ciliary transmembrane protein, Peripherin.

14 export and translation, but had no effect on peripherin.

15 g for splice site mutation c.828+3A>T in the peripherin 2 (PRPH2) gene should be a high priority in f

16 Peripherin 2 (Prph2) is a photoreceptor tetraspanin, and

17 Mutations in peripherin 2 (PRPH2), also known as retinal degeneration

18 pigmentosa, lacks a functional gene encoding peripherin 2.

19 Peripherin-2 (also known as peripherin/rds), a photorece

20 hotoreceptor-specific membrane glycoprotein, peripherin-2 (also known as peripherin/rds), which is in

21 Mutations in the photoreceptor-specific gene peripherin-2 (PRPH-2, also known as retinal degeneration

22 ypeptide comprising the C-terminus of bovine peripherin-2 (R284-G346) binds to the membrane mimetic,

23 ation of EGFP-PDE6C with the disc rim marker peripherin-2 and endogenous frog PDE6.

24 of intracellular membrane fusion induced by peripherin-2 and the mechanism of enveloped viral fusion

25 Previously published data showed that both peripherin-2 and viral fusion proteins are transmembrane

26 lular retinal rod outer segment disk protein peripherin-2 binds to membranes, adopts a helical confor

27 Furthermore, the fusion-active sequence of peripherin-2 exhibits a sequence motif that matches the

28 In this study, we show that peripherin-2 forms a complex with melanoregulin (MREG),

29 that this newly identified protein regulates peripherin-2 function.

30 MREG colocalized with peripherin-2 in WT (CB6F1/J) and in rds+/- retinas.

31 the C terminus of human, bovine, and murine peripherin-2 is an amphiphilic fusion peptide domain (re

32 s et al. show that peripherin (also known as peripherin-2 or peripherin-2/rds) diverts membrane traff

33 Peripherin-2 protein was produced in yeast and analyzed

34 the structure of the transmembrane domain of peripherin-2 was constructed.

35 e, P296T, P296A, P296L, and P296E mutants of peripherin-2 were expressed as His6-tagged full-length p

36 nts or contain the associated markers ROM-1, peripherin-2, and RP1.

37 Peripherin-2, the product of the rds gene, is a tetraspa

38 owed formation of a complex between MREG and peripherin-2-ROM-1 hetero-oligomers.

39 in, peripherin/rds, rds/peripherin, rds, and peripherin-2.) Peripherin/rds appears to act as a struct

40 By contrast, anti-peripherin-2/rds antibody, Xper5A11, labeled the closed

41 significantly strengthens the evidence that peripherin-2/rds functions directly to shape the high-cu

42 Peripherin-2/rds is required for biogenesis of vertebrat

43 at peripherin (also known as peripherin-2 or peripherin-2/rds) diverts membrane traffic to photorecep

44 gests that within the context of full-length peripherin-2/rds, partitioning would most likely occur a

45 ations in the photoreceptor tetraspanin gene peripherin-2/retinal degeneration slow (PRPH2/RDS) cause

46 tnatal and adult mice, via immunostaining of peripherin (a marker of thinly myelinated and unmyelinat

47 Peripherin, a neuronal intermediate filament protein imp

48 Peripherin also coassembles into a single network of fil

49 Salinas et al. show that peripherin (also known as peripherin-2 or peripherin-2/r

50 mediate filament proteins (alpha-internexin, peripherin) also accumulated in these spheroids.

51 e absence of retinal degeneration slow (RDS)/peripherin, an essential outer segment structural protei

52 al degeneration slow or rds gene encodes rds/peripherin, an integral membrane glycoprotein in the out

53 are the result of regulatory changes to the peripherin ancestral gene along each lineage.

54 In contrast, the IFs peripherin and alpha-internexin are preferentially local

55 Recent studies have identified peripherin and ATF-3 molecules as crucial for neurite ou

56 Since peripherin and BPAG1-n also display similar expression p

57 as been found that peripherin-siRNA depletes peripherin and inhibits the initiation, extension, and m

58 5.6 mum(2)) and predominantly colabeled with peripherin and isolectin B4 markers of unmyelinated C-fi

59 Peripherin and its homologue ROM1 are retina-specific me

60 nt (nIF) protein family, which also includes peripherin and neurofilament (NF) triplet proteins.

61 was similarly involved in the degradation of peripherin and neurofilament IF proteins in neurons.

62 fected into vimentin(+) SW13 cells, and with peripherin and neurofilament proteins when transfected i

63 neurons, endogenous Nes-S co-assembles with peripherin and neurofilament proteins.

64 mers, have supported a widely held view that peripherin and neurofilament triplets form separate fila

65 The relative proportions of peripherin and NF-M increased above normal, whereas prop

66 The increases in peripherin and NF-M mRNAs were accompanied by increases

67 in both S and L/M cones, but S cones express peripherin and opsin 1 to 3 weeks before neighboring L/M

68 Peripherin and ROM1 interact in vivo and are predicted t

69 200 kDa neurofilament (NF-H) epitopes, NPY, peripherin and synapsin I, but not to hypophosphorylated

70 These data show that peripherin and the neurofilament proteins are functional

71 After crush, peripherin and ubiquitin levels remained also significan

72 uron-specific intermediate filament protein, peripherin, and by RA-stimulated extension of neuritic p

73 positive for 200 kDa neurofilament subunit, peripherin, and carbonic anhydrase.

74 to induce the expression of sodium channels, peripherin, and Thy-1 in PC12 cells because all three of

75 lectively increased transport of beta-actin, peripherin, and vimentin mRNAs from the cell body into t

76 adult calvaria, the highest concentration of peripherin- and CGRP-labeled fibers was found in sutures

77 he RNA for which binds hnRNP K, with that of peripherin, another intermediate filament protein, the R

78 Neurofilaments and peripherin appear to play some part in motor neuron dege

79 Peripherin appears concomitantly with opsin in both S an

80 d the classification of XIF3, plasticin, and peripherin as orthologs (products of the same genetic lo

81 Transgenic peripherin autoreactive B-lymphocytes infiltrate NOD pan

82 The unmyelinated innervation had detectable peripherin but not 160 kDa or 200 kDa neurofilament subu

83 ssed in many neurons that contain endogenous peripherin but was activated after injury.

84 n of mouse BPAG1 interacts specifically with peripherin, but in contrast to a previous study, mouse B

85 Peripherin, but not ROM1, is glycosylated and we examine

86 Over 100 pathogenic mutations in peripherin cause inherited rod- and cone-related dystrop

87 Canine RDS/peripherin cDNA was cloned and sequenced after reverse-t

88 ly deleting NFL in mice dramatically reduces peripherin content in sciatic axons.

89 ee of GFP expression by neurofilament(+) and peripherin(+) DRG neurons was similar.

90 in-like growth factor II, guanine deaminase, peripherin, early growth response 1, soluble guanylate c

91 Only neurons without peripherin epitopes, putative type I neurons, showed syn

92 Peripherin exhibits a distribution pattern identical to

93 proteins/protein complexes in extracts from peripherin-expressing and nonexpressing tissues.

94 GDNF promoted the development of peripherin-expressing neurons (E12 >> E14-16) and expres

95 ed synergistic survival effects; survival of peripherin-expressing neurons was purely additive.

96 Dramatic increases in peripherin expression have been shown to coincide with t

97 e element (centered at -173), which prevents peripherin expression in undifferentiated PC12 cells, an

98 in other successfully regenerating systems, peripherin expression increased rapidly after injury and

99 ctive and that neuron-specific regulation of peripherin expression may be due to interaction with dis

100 The canine RDS/peripherin gene (CFRDSP) has been excluded as a candidat

101 Thus, accurate cell type-specific peripherin gene expression in the PNS depends on element

102 king region, are required for activating the peripherin gene in response to nerve injury.

103 and revealed a missense mutation in the RDS/peripherin gene leading to a Tyr141Cys substitution.

104 PMSG was applied to the RDS/peripherin gene of 16 individuals from a family exhibiti

105 mutant peripherin transgene under control of peripherin gene regulatory sequences.

106 The peripherin gene, encoding a neuron-specific intermediate

107 (rds) mice which carry a mutation in the rds/peripherin gene.

108 e identified two regulatory sequences of the peripherin gene: a proximal negative element (centered a

109 pecific or injury-specific expression of the peripherin gene; however, the remaining intron, intron 1

110 th RP in humans, such as RHO, PDE6B, and RDS/peripherin, have been excluded.

111 esults of these experiments demonstrate that peripherin IF are critical determinants of the overall s

112 re organism in vivo, we disrupted endogenous peripherin IF in small-sized dorsal root ganglion (DRG)

113 in (10(-4) M) had no effect on the length of peripherin-immunoreactive olfactory fibers or LHRH cell

114 60 to -2308) necessary for full induction of peripherin in differentiated PC12 cells.

115 S and is activated after nerve injury is the peripherin intermediate filament gene, but little is kno

116 vo by studying the synthesis and assembly of peripherin intermediate filaments (IFs).

117 ather than forming an independent structure, peripherin is a subunit of neurofilaments in the adult P

118 Peripherin is a type III intermediate filament (IF) prot

119 system, a fifth neuronal IF protein known as peripherin is also present.

120 , despite a postnatal decline in expression, peripherin is as abundant as the triplet in the adult PN

121 Peripherin is cotranslationally assembled into insoluble

122 on of the intermediate filament (IF) protein peripherin is initiated during development at the time o

123 This new function of peripherin is performed independently from its well-esta

124 In dt mice, peripherin is present in axonal swellings of degeneratin

125 terns in the nervous system, we suggest that peripherin is the specific interaction partner of BPAG1-

126 ith markers of primary afferent nociceptors, peripherin, isolectin B4, and substance P, and markers o

127 Peripherin labeling of outer segments was nonuniform, an

128 Peripherin labels bouton-only afferents.

129 iption of these molecules and that a greater peripherin mRNA expression in injured cutaneous neurons

130 The results show that peripherin mRNA-containing particles (messenger ribonucl

131 Many peripherin mRNPs contain multiple mRNAs, possibly amplif

132 ults show that the motility and targeting of peripherin mRNPs, their translational control, and the a

133 nsgenic mice heterozygous for a specific rds/peripherin mutation (P216L) have lower DHA levels in the

134 (+/-) mutations, and mice with the P216L rds/peripherin mutation on the rds(+/-) background were main

135 Mice heterozygous for the spontaneous rds/peripherin mutation or mice carrying the P216L mutation

136 Moreover, peripherin mutations has been shown to disrupt the neuro

137 inent p62-immunoreactive, alphaS-, NFL-, and peripherin-negative inclusions.

138 n diseases, both large SMI-32(+) neurons and peripherin(+) neurons are selectively damaged by prolong

139 ed index of the motor neuron population) and peripherin(+) neurons share with large SMI-32(+) neurons

140 ity is provided by the observation that most peripherin(+) neurons, like large SMI-32(+) neurons, are

141 neurites, which have NFs made exclusively of peripherin, NFs were unaffected, but in the shaft of old

142 GP 9.5) as well as more specialized markers (peripherin, nNOS) in ES-NS, both at the transcriptional

143 cone opsin and either a structural protein (peripherin) or a phototransduction cascade protein (alph

144 The expression of a peripherin ortholog in Xenopus glia may represent either

145 eripherin/rds is also known as photoreceptor peripherin, peripherin/rds, rds/peripherin, rds, and per

146 ells showed the presence of neuronal markers peripherin, PGP9.5, HuD, tau, synaptic marker synaptophy

147 ), glutathione S-transferase mu2, cyclin D2, peripherin, plasma glutathione peroxidase, methyl CpG-bi

148 evelopment and regeneration, suggesting that peripherin plays an important role in axon formation.

149 Most of these neurons are peripherin positive and produce C fibers, although a sma

150 sed numbers of presumptive nociceptor axons (peripherin-positive axons containing calcitonin gene-rel

151 The numbers of peripherin-positive fibres also increased in the mucosal

152 Thus, XIF3, plasticin, and peripherin probably should be referred to as Xenopus, fi

153 bit accumulation of neurofilament (NF-L) and peripherin (PRPH) protein and formation of PRPH aggregat

154 the NOD mouse model to the neuronal protein peripherin (PRPH), a target Ag of islet-infiltrating B c

155 encoding the type III intermediate filament peripherin (Prph).

156 RDS encodes rds/peripherin (rds), a 36-kDa glycoprotein in the rims of r

157 The rds gene encodes rds/peripherin (rds), a membrane glycoprotein in the rims of

158 hotoreceptor peripherin, peripherin/rds, rds/peripherin, rds, and peripherin-2.) Peripherin/rds appea

159 t disk membrane proteins, such as rhodopsin, Peripherin-rds, Rom-1, and Abca4, but significantly disr

160 transgenic line expressing the normal mouse peripherin/Rds (NMP) was crossed with a higher-expresser

161 a transgenic mouse line expressing wild-type peripherin/rds (NMP), the authors evaluated the critical

162 Peripherin/rds (P/rds) is a disk rim protein that assemb

163 Peripherin/rds (P/rds) is a membrane glycoprotein essent

164 st membrane renewal, for which rhodopsin and peripherin/rds (P/rds) play critical roles.

165 ontribute to OS organization in concert with peripherin/rds (P/rds), a retinal tetraspanin.

166 Peripherin/rds (p/rds), an integral membrane protein fro

167 The gene product, peripherin/rds (P/rds), is a member of the tetraspanin p

168 Peripherin/rds (P/rds), the product of the retinal degen

169 enes analyzed, including rhodopsin (RP4) and peripherin/RDS (RP7), for the four adRP loci RP1, RP9, R

170 5' and 3' untranslated regions of the skate peripherin/rds (srds) cDNA were isolated by the rapid am

171 d caveolin; surprisingly, the fusion protein peripherin/rds (where rds is retinal degeneration slow),

172 C-terminally truncated peripherin/rds (Xper38)-GFP chimeric protein sorting was

173 SRDS protein is 54.5% identical with peripherin/rds across species.

174 ion of nearly 2 mol of phosphate per mole of peripherin/rds and a concomitant shift in the isoelectri

175 rbation are produced by inherited defects in peripherin/rds and contribute to the observed heterogene

176 The proteins peripherin/rds and rom-1 form a protein complex in the r

177 Peripherin/rds and rom-1 have structural roles in morpho

178 Peripherin/rds and rom-1 localized to this ciliary membr

179 Peripherin/rds and rom-1 trafficking is maintained in rh

180 Peripherin/rds and rom-1 trafficking were studied in sev

181 outer segment formation and distribution of peripherin/rds and rom-1 were examined by immunohistoche

182 Peripherin/RDS and ROM-1, two structural proteins that c

183 that likely reflects the tight regulation of peripherin/rds and the need for two functional alleles t

184 rds, rds/peripherin, rds, and peripherin-2.) Peripherin/rds appears to act as a structural element in

185 ations in the photoreceptor-specific protein peripherin/rds are associated with multiple retinal dise

186 s of the photoreceptor disc membrane protein peripherin/rds by analysis of the homologue in a primiti

187 Overexpression of peripherin/rds caused no detectable adverse effects on r

188 inal cDNA library was screened using a mouse peripherin/rds clone.

189 Uptake of rhodopsin and peripherin/rds coincided with the overgrowth of the evag

190 from samples prepared in the dark (13%), but peripherin/rds could not be detected in either preparati

191 n finds its way into the plasma membrane but peripherin/rds does not, remaining behind in vesicles.

192 A positive correlation was observed between peripherin/rds expression levels and the structural and

193 ogenetic analysis places SRDS at the base of peripherin/rds family and near the division of that grou

194 ic procedures for the purification of native peripherin/rds from bovine photoreceptor cell outer segm

195 Analysis of homogeneous peripherin/rds from the second procedure showed that exp

196 The GFP-peripherin/rds fusion proteins did not immunoprecipitate

197 Presence of GFP-peripherin/rds fusions correlated with disrupted incisur

198 maintenance of disk membrane structure, with peripherin/rds gene mutations resulting in photoreceptor

199 Heterozygotes with these different peripherin/RDS gene mutations showed variation in clinic

200 The coding regions of the peripherin/RDS gene were sequenced in each patient.

201 neration were found to have mutations in the peripherin/RDS gene.

202 t distinct loci, including the rhodopsin and peripherin/RDS genes as well as unidentified genes on ch

203 represents the skate orthologue of mammalian peripherin/rds genes.

204 ause of a null mutation in the gene encoding peripherin/rds has been thought to be the primary defect

205 photoreceptor cell's plasma membrane, while peripherin/rds immunolabeling occurs within cytoplasmic

206 These results demonstrate the necessity for peripherin/rds in ROS membrane fusion, specifically the

207 describes novel roles for the C terminus of peripherin/rds in targeting and maintaining ROS structur

208 ed us to characterize the functional role of peripherin/rds in the maintenance of rod outer segment (

209 ion, we found that recombination of purified peripherin/rds into lipid vesicles increased membrane fu

210 (Peripherin/rds is also known as photoreceptor peripherin

211 Peripherin/rds is an integral membrane protein required

212 ntegrity of the second intradiscal domain of peripherin/RDS is critical for normal macular cone struc

213 ly, both insert into the plasma membrane but peripherin/rds is recycled into cytoplasmic vesicles.

214 Total peripherin/rds levels in the retina were modulated by cr

215 A GFP fusion containing residues 317-336 of peripherin/rds localized uniformly to disk membranes.

216 uter segment supports previous evidence that peripherin/rds mice form homotetramers for outer segment

217 l coherence tomography in four patients with peripherin/RDS mutations and 27 age-similar healthy subj

218 peripherin/RDS mutations produced diffuse AF abnormaliti

219 this study suggest that human patients with peripherin/rds mutations, or other mutations that result

220 hors evaluated the critical in vivo level of peripherin/rds needed to maintain photoreceptor structur

221 sion proteins did not immunoprecipitate with peripherin/rds or rom-1, suggesting this region does not

222 RG function and assessed the consequences of peripherin/rds overexpression in both rods and cones by

223 a series of overlapping synthetic C-terminal peripherin/rds peptides to mediate model membrane fusion

224 Peripherin/rds plays an essential role in the maintenanc

225 Peripherin/rds plays an essential role in the morphogene

226 Peripherin/rds protein levels in NMP homozygotes were ap

227 which dictates well-regulated expression of peripherin/rds protein levels.

228 rated appropriate localization of transgenic peripherin/rds protein to the disc rim region of photore

229 In conclusion, studies with purified peripherin/rds reveal that the protein undergoes light-d

230 id not prevent the entrance of rhodopsin and peripherin/rds to the membrane evaginations believed to

231 Peripherin/rds transport and localization are polarized

232 During one procedure, peripherin/rds was copurified in association with rom-1

233 In developing wild-type photoreceptors, peripherin/rds was detected exclusively in the distal ti

234 A new homologue of peripherin/rds was identified from the skate retinal cDN

235 reorganization of disk membranes, opsin and peripherin/rds were fused to a fluorescent protein, Dend

236 Heterozygous mutations in peripherin/RDS were predicted to affect protein structur

237 s injected subretinally, for transduction of peripherin/rds(+/)(-) transgenic mice that carry the P21

238 Peripherin-2 (also known as peripherin/rds), a photoreceptor specific tetraspanin pr

239 ne glycoprotein, peripherin-2 (also known as peripherin/rds), which is inserted into the rims of phot

240 nd cone-specific proteins such as phosducin, peripherin/rds, and ROM1.

241 Rhodopsin, peripherin/RDS, and TIMP3 genes were screened for mutati

242 ted in the coding sequence of the rhodopsin, peripherin/RDS, and TIMP3 genes.

243 f degeneration, rod and cone opsins, but not peripherin/RDS, exhibited prominent ectopic localization

244 orphogenesis is reliant upon the presence of peripherin/rds, hereafter termed Rds.

245 ecific integral membrane proteins, opsin and peripherin/rds, in rod photoreceptors.

246 s is also known as photoreceptor peripherin, peripherin/rds, rds/peripherin, rds, and peripherin-2.)

247 nsgenic protein in the presence of wild-type peripherin/rds, using the monoclonal antibody 3B6.

248 ations regarding therapeutic intervention in peripherin/rds-associated retinal diseases.

249 g is cargo-specific and was not observed for peripherin/rds-Dendra2.

250 protein, that may play a regulatory role in peripherin/rds-dependent fusion.

251 sma membrane (R18-PM) with disk membranes or peripherin/rds-enriched large unilammellar vesicles (LUV

252 transgenic Xenopus, a C-terminally truncated peripherin/rds-GFP fusion predominantly localized to its

253 The presence of truncated peripherin/rds-GFP in the outer segment supports previou

254 al and functional rescue in animal models of peripherin/rds-induced retinal degeneration have not bee

255 ethods in wild-type, rhodopsin-knockout, and peripherin/rds-knockout mice.

256 ot been fully characterized, the presence of peripherin/rds-positive vesicles adjacent to the striate

257 the NMP protein when compared with wild-type peripherin/rds.

258 oteins for other proteins that interact with peripherin/rds.

259 es (LUVs) is inhibited upon trypsinolysis of peripherin/rds.

260 re rapid fusion detected with phosphorylated peripherin/rds.

261 effect on the traffic of rhodopsin, Rom1 or peripherin/rds; however, the retinal guanylate cyclases

262 es, and mammals (called XIF3, plasticin, and peripherin, respectively) that are classified as type II

263 referred to as Xenopus, fish, and mammalian peripherin, respectively.

264 Peripherin/retinal degeneration slow (rds) and rom-1 are

265 ophoresis, we confirmed that the core native peripherin-ROM1 complex exists as a tetramer.

266 d methodology for high yield purification of peripherin-ROM1 from bovine retinas that permitted its b

267 Finally, we demonstrated that peripherin-ROM1 tetramers induce membrane curvature when

268 candidate RP genes, including rhodopsin, RDS/peripherin, RP2, and RPGR, were also examined in this pr

269 function is fulfilled by a separate part of peripherin's molecule.

270 In this study, it has been found that peripherin-siRNA depletes peripherin and inhibits the in

271 RDS/peripherin-specific restriction fragment length polymorp

272 Peripherin staining was irregular and disorganized.

273 olecular-mass neurofilament subunit (NFL) or peripherin staining.

274 The concomitant appearance of opsin and peripherin strongly suggests roles in promoting the stru

275 ic structural proteins (e.g. neurofilaments, peripherin, Talpha1-tubulin) have revealed potential reg

276 The earlier peak expression of peripherin than the triplet during brain development and

277 e effects of a transgene encoding normal rds/peripherin that had integrated into the X-chromosome in

278 ysis to include the four light NF subunits - peripherin, the light NF triplet protein (NF-L), and two

279 n transgenic mice via expression of a mutant peripherin transgene under control of peripherin gene re

280 critical, mice were generated that expressed peripherin transgenes lacking different introns.

281 As for the cytoskeletal proteins beta-actin, peripherin, vimentin, gamma-tropomyosin 3, and cofilin 1

282 Two new homologs of rds/peripherin were discovered: crds1 and crds2.

283 segment proteins (the cGMP-gated channel and peripherin) were examined and found to be sorted normall

284 yclic GMP phosphodiesterase (PDE6B), and RDS/peripherin, were excluded.

285 presents the chick ortholog of mammalian rds/peripherin, whereas crds2 is a more distant homolog.

286 s is suppressed by the disc-specific protein peripherin, which enables retained ectosomes to be morph

287 sed throughout regeneration, whereas that of peripherin, which is abundant in developing axons, incre

288 ression of the intermediate filament protein peripherin, which is associated with CNS neuronal injury

289 bled a mutant rds transgene that encodes rds/peripherin with a P216L substitution.