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1 holine, calcitonin gene-related peptide, and peripherin).
2 ronal type III intermediate filament protein peripherin.
3 ly labeled to detect alpha9, calretinin, and peripherin.
4 TrkC, and RET and the sensory neurofilament peripherin.
5 on endings, are not labeled by calretinin or peripherin.
6 t contain the alpha9 nAChR but did stain for peripherin.
7 tein (GFAP); and the nerve structural marker peripherin.
8 in NOD mice recognizing the neuronal antigen peripherin.
9 the absence of the outer segment protein rds/peripherin.
10 2 protein is 54%, identical to mammalian rds/peripherin.
11 , and a reduction in the level of normal rds/peripherin.
12 ucine (P216L) amino acid substitution in rds/peripherin.
13 nied by gain of the tetraspanin rim protein, peripherin.
14 photoreceptor ciliary transmembrane protein, Peripherin.
15 export and translation, but had no effect on peripherin.
17 g for splice site mutation c.828+3A>T in the peripherin 2 (PRPH2) gene should be a high priority in f
21 alfunctions of the LE.SIGNIFICANCE STATEMENT Peripherin 2 (PRPH2) is a tetraspanin protein abundantly
27 hotoreceptor-specific membrane glycoprotein, peripherin-2 (also known as peripherin/rds), which is in
28 Mutations in the photoreceptor-specific gene peripherin-2 (PRPH-2, also known as retinal degeneration
29 ypeptide comprising the C-terminus of bovine peripherin-2 (R284-G346) binds to the membrane mimetic,
31 of intracellular membrane fusion induced by peripherin-2 and the mechanism of enveloped viral fusion
32 Previously published data showed that both peripherin-2 and viral fusion proteins are transmembrane
33 lular retinal rod outer segment disk protein peripherin-2 binds to membranes, adopts a helical confor
34 Furthermore, the fusion-active sequence of peripherin-2 exhibits a sequence motif that matches the
38 the C terminus of human, bovine, and murine peripherin-2 is an amphiphilic fusion peptide domain (re
39 s et al. show that peripherin (also known as peripherin-2 or peripherin-2/rds) diverts membrane traff
40 ereby normal rods express a modest excess of peripherin-2 over the amount required for complete disc
43 e, P296T, P296A, P296L, and P296E mutants of peripherin-2 were expressed as His6-tagged full-length p
47 in, peripherin/rds, rds/peripherin, rds, and peripherin-2.) Peripherin/rds appears to act as a struct
48 that the photoreceptor-specific tetraspanin, peripherin-2/rds (P/rds), may contribute to the highly c
50 significantly strengthens the evidence that peripherin-2/rds functions directly to shape the high-cu
52 at peripherin (also known as peripherin-2 or peripherin-2/rds) diverts membrane traffic to photorecep
53 gests that within the context of full-length peripherin-2/rds, partitioning would most likely occur a
54 ations in the photoreceptor tetraspanin gene peripherin-2/retinal degeneration slow (PRPH2/RDS) cause
55 tnatal and adult mice, via immunostaining of peripherin (a marker of thinly myelinated and unmyelinat
60 e absence of retinal degeneration slow (RDS)/peripherin, an essential outer segment structural protei
61 al degeneration slow or rds gene encodes rds/peripherin, an integral membrane glycoprotein in the out
66 as been found that peripherin-siRNA depletes peripherin and inhibits the initiation, extension, and m
67 5.6 mum(2)) and predominantly colabeled with peripherin and isolectin B4 markers of unmyelinated C-fi
70 was similarly involved in the degradation of peripherin and neurofilament IF proteins in neurons.
71 fected into vimentin(+) SW13 cells, and with peripherin and neurofilament proteins when transfected i
73 mers, have supported a widely held view that peripherin and neurofilament triplets form separate fila
76 in both S and L/M cones, but S cones express peripherin and opsin 1 to 3 weeks before neighboring L/M
78 200 kDa neurofilament (NF-H) epitopes, NPY, peripherin and synapsin I, but not to hypophosphorylated
81 uron-specific intermediate filament protein, peripherin, and by RA-stimulated extension of neuritic p
83 to induce the expression of sodium channels, peripherin, and Thy-1 in PC12 cells because all three of
84 lectively increased transport of beta-actin, peripherin, and vimentin mRNAs from the cell body into t
85 adult calvaria, the highest concentration of peripherin- and CGRP-labeled fibers was found in sutures
86 he RNA for which binds hnRNP K, with that of peripherin, another intermediate filament protein, the R
89 d the classification of XIF3, plasticin, and peripherin as orthologs (products of the same genetic lo
91 The unmyelinated innervation had detectable peripherin but not 160 kDa or 200 kDa neurofilament subu
93 n of mouse BPAG1 interacts specifically with peripherin, but in contrast to a previous study, mouse B
99 in-like growth factor II, guanine deaminase, peripherin, early growth response 1, soluble guanylate c
106 e element (centered at -173), which prevents peripherin expression in undifferentiated PC12 cells, an
107 in other successfully regenerating systems, peripherin expression increased rapidly after injury and
108 ctive and that neuron-specific regulation of peripherin expression may be due to interaction with dis
112 and revealed a missense mutation in the RDS/peripherin gene leading to a Tyr141Cys substitution.
117 e identified two regulatory sequences of the peripherin gene: a proximal negative element (centered a
118 pecific or injury-specific expression of the peripherin gene; however, the remaining intron, intron 1
120 esults of these experiments demonstrate that peripherin IF are critical determinants of the overall s
121 re organism in vivo, we disrupted endogenous peripherin IF in small-sized dorsal root ganglion (DRG)
122 in (10(-4) M) had no effect on the length of peripherin-immunoreactive olfactory fibers or LHRH cell
124 S and is activated after nerve injury is the peripherin intermediate filament gene, but little is kno
126 ather than forming an independent structure, peripherin is a subunit of neurofilaments in the adult P
129 , despite a postnatal decline in expression, peripherin is as abundant as the triplet in the adult PN
131 on of the intermediate filament (IF) protein peripherin is initiated during development at the time o
134 terns in the nervous system, we suggest that peripherin is the specific interaction partner of BPAG1-
135 ith markers of primary afferent nociceptors, peripherin, isolectin B4, and substance P, and markers o
138 iption of these molecules and that a greater peripherin mRNA expression in injured cutaneous neurons
141 ults show that the motility and targeting of peripherin mRNPs, their translational control, and the a
142 nsgenic mice heterozygous for a specific rds/peripherin mutation (P216L) have lower DHA levels in the
143 (+/-) mutations, and mice with the P216L rds/peripherin mutation on the rds(+/-) background were main
144 Mice heterozygous for the spontaneous rds/peripherin mutation or mice carrying the P216L mutation
147 n diseases, both large SMI-32(+) neurons and peripherin(+) neurons are selectively damaged by prolong
148 ed index of the motor neuron population) and peripherin(+) neurons share with large SMI-32(+) neurons
149 ity is provided by the observation that most peripherin(+) neurons, like large SMI-32(+) neurons, are
150 neurites, which have NFs made exclusively of peripherin, NFs were unaffected, but in the shaft of old
151 GP 9.5) as well as more specialized markers (peripherin, nNOS) in ES-NS, both at the transcriptional
152 cone opsin and either a structural protein (peripherin) or a phototransduction cascade protein (alph
154 eripherin/rds is also known as photoreceptor peripherin, peripherin/rds, rds/peripherin, rds, and per
155 ells showed the presence of neuronal markers peripherin, PGP9.5, HuD, tau, synaptic marker synaptophy
156 ), glutathione S-transferase mu2, cyclin D2, peripherin, plasma glutathione peroxidase, methyl CpG-bi
157 evelopment and regeneration, suggesting that peripherin plays an important role in axon formation.
159 sed numbers of presumptive nociceptor axons (peripherin-positive axons containing calcitonin gene-rel
162 bit accumulation of neurofilament (NF-L) and peripherin (PRPH) protein and formation of PRPH aggregat
163 the NOD mouse model to the neuronal protein peripherin (PRPH), a target Ag of islet-infiltrating B c
167 hotoreceptor peripherin, peripherin/rds, rds/peripherin, rds, and peripherin-2.) Peripherin/rds appea
168 t disk membrane proteins, such as rhodopsin, Peripherin-rds, Rom-1, and Abca4, but significantly disr
169 transgenic line expressing the normal mouse peripherin/Rds (NMP) was crossed with a higher-expresser
170 a transgenic mouse line expressing wild-type peripherin/rds (NMP), the authors evaluated the critical
178 enes analyzed, including rhodopsin (RP4) and peripherin/RDS (RP7), for the four adRP loci RP1, RP9, R
179 5' and 3' untranslated regions of the skate peripherin/rds (srds) cDNA were isolated by the rapid am
180 d caveolin; surprisingly, the fusion protein peripherin/rds (where rds is retinal degeneration slow),
183 ion of nearly 2 mol of phosphate per mole of peripherin/rds and a concomitant shift in the isoelectri
184 rbation are produced by inherited defects in peripherin/rds and contribute to the observed heterogene
190 outer segment formation and distribution of peripherin/rds and rom-1 were examined by immunohistoche
192 that likely reflects the tight regulation of peripherin/rds and the need for two functional alleles t
193 rds, rds/peripherin, rds, and peripherin-2.) Peripherin/rds appears to act as a structural element in
194 ations in the photoreceptor-specific protein peripherin/rds are associated with multiple retinal dise
195 s of the photoreceptor disc membrane protein peripherin/rds by analysis of the homologue in a primiti
199 from samples prepared in the dark (13%), but peripherin/rds could not be detected in either preparati
200 n finds its way into the plasma membrane but peripherin/rds does not, remaining behind in vesicles.
201 A positive correlation was observed between peripherin/rds expression levels and the structural and
202 ogenetic analysis places SRDS at the base of peripherin/rds family and near the division of that grou
203 ic procedures for the purification of native peripherin/rds from bovine photoreceptor cell outer segm
207 maintenance of disk membrane structure, with peripherin/rds gene mutations resulting in photoreceptor
211 t distinct loci, including the rhodopsin and peripherin/RDS genes as well as unidentified genes on ch
213 ause of a null mutation in the gene encoding peripherin/rds has been thought to be the primary defect
214 photoreceptor cell's plasma membrane, while peripherin/rds immunolabeling occurs within cytoplasmic
215 These results demonstrate the necessity for peripherin/rds in ROS membrane fusion, specifically the
216 describes novel roles for the C terminus of peripherin/rds in targeting and maintaining ROS structur
217 ed us to characterize the functional role of peripherin/rds in the maintenance of rod outer segment (
218 ion, we found that recombination of purified peripherin/rds into lipid vesicles increased membrane fu
221 ntegrity of the second intradiscal domain of peripherin/RDS is critical for normal macular cone struc
222 ly, both insert into the plasma membrane but peripherin/rds is recycled into cytoplasmic vesicles.
224 A GFP fusion containing residues 317-336 of peripherin/rds localized uniformly to disk membranes.
225 uter segment supports previous evidence that peripherin/rds mice form homotetramers for outer segment
226 l coherence tomography in four patients with peripherin/RDS mutations and 27 age-similar healthy subj
228 this study suggest that human patients with peripherin/rds mutations, or other mutations that result
229 hors evaluated the critical in vivo level of peripherin/rds needed to maintain photoreceptor structur
230 sion proteins did not immunoprecipitate with peripherin/rds or rom-1, suggesting this region does not
231 RG function and assessed the consequences of peripherin/rds overexpression in both rods and cones by
232 a series of overlapping synthetic C-terminal peripherin/rds peptides to mediate model membrane fusion
237 rated appropriate localization of transgenic peripherin/rds protein to the disc rim region of photore
239 id not prevent the entrance of rhodopsin and peripherin/rds to the membrane evaginations believed to
242 In developing wild-type photoreceptors, peripherin/rds was detected exclusively in the distal ti
244 reorganization of disk membranes, opsin and peripherin/rds were fused to a fluorescent protein, Dend
246 s injected subretinally, for transduction of peripherin/rds(+/)(-) transgenic mice that carry the P21
248 ne glycoprotein, peripherin-2 (also known as peripherin/rds), which is inserted into the rims of phot
252 f degeneration, rod and cone opsins, but not peripherin/RDS, exhibited prominent ectopic localization
255 s is also known as photoreceptor peripherin, peripherin/rds, rds/peripherin, rds, and peripherin-2.)
260 sma membrane (R18-PM) with disk membranes or peripherin/rds-enriched large unilammellar vesicles (LUV
261 transgenic Xenopus, a C-terminally truncated peripherin/rds-GFP fusion predominantly localized to its
263 al and functional rescue in animal models of peripherin/rds-induced retinal degeneration have not bee
265 ot been fully characterized, the presence of peripherin/rds-positive vesicles adjacent to the striate
270 effect on the traffic of rhodopsin, Rom1 or peripherin/rds; however, the retinal guanylate cyclases
271 Together, this study presents a new model of peripherin-reactive B lymphocyte-dependent autoimmune ne
273 es, and mammals (called XIF3, plasticin, and peripherin, respectively) that are classified as type II
277 d methodology for high yield purification of peripherin-ROM1 from bovine retinas that permitted its b
279 candidate RP genes, including rhodopsin, RDS/peripherin, RP2, and RPGR, were also examined in this pr
285 The concomitant appearance of opsin and peripherin strongly suggests roles in promoting the stru
286 ic structural proteins (e.g. neurofilaments, peripherin, Talpha1-tubulin) have revealed potential reg
288 e effects of a transgene encoding normal rds/peripherin that had integrated into the X-chromosome in
289 ysis to include the four light NF subunits - peripherin, the light NF triplet protein (NF-L), and two
290 n transgenic mice via expression of a mutant peripherin transgene under control of peripherin gene re
292 As for the cytoskeletal proteins beta-actin, peripherin, vimentin, gamma-tropomyosin 3, and cofilin 1
294 segment proteins (the cGMP-gated channel and peripherin) were examined and found to be sorted normall
296 presents the chick ortholog of mammalian rds/peripherin, whereas crds2 is a more distant homolog.
297 s is suppressed by the disc-specific protein peripherin, which enables retained ectosomes to be morph
298 sed throughout regeneration, whereas that of peripherin, which is abundant in developing axons, incre
299 ression of the intermediate filament protein peripherin, which is associated with CNS neuronal injury