ライフサイエンスコーパス: PC1

1 PC1 ('binge-eating'), accounting for 38% of variation, c

2 PC1 also complexes with Rab8; knockdown of trafficking r

3 PC1 and Pacsin 2 co-localize on the lamellipodia of migr

4 PC1 and Pacsin 2-deficient kidney epithelial cells migra

5 PC1 and PC2 are secreted on urinary exosome-like vesicle

6 PC1 binds a multimeric protein complex consisting of sev

7 PC1 binds to a 107-residue fragment containing the alpha

8 PC1 explained 32.7% of the variation and depicted vertic

9 PC1 has a predicted molecular mass of ~460 kDa comprisin

10 PC1 has been shown to form a complex with PC2, and the s

11 PC1 is a complex polytopic membrane protein expressed in

12 PC1 is an integral membrane protein, which has been impl

13 PC1 must also be proteolytically cleaved at a GPS site f

14 PC1 separated Basmati from the other two cultivars and P

15 PC1 surface localization in GANAB(-/-) cells was rescued

16 PC1 was associated with a SNP near PAX5 (P = 0.01).

17 PC1 was previously shown to slow cell proliferation and

18 PC1&PC2 independently predicted 90-day mortality (ORs 2.

19 PC1(hi) cells were also more efficient than PC1(lo) cell

20 PC1(mussel) and PC1(discharge) were closely linked to re

21 PC1/3 is an endoprotease that processes many prohormones

22 PC1/3 KO mice have an enlarged spleen with marked disorg

23 by expression of plasma cell alloantigen 1 (PC1), also known as ectonucleotide pyrophosphatase phosp

24 duced expression of prohormone convertase 1 (PC1).

25 Polycystin-1 (PC1) and -2 (PC2), the two ADPKD gene products, are larg

26 oteins encoded by these genes, polycystin-1 (PC1) and polycystin-2 (PC2), form a plasma membrane rece

27 The ADPKD proteins, termed as polycystin-1 (PC1) and polycystin-2 (PC2), interact via their C-termin

28 in PKD1 or PKD2, which encode polycystin-1 (PC1) and polycystin-2 (PC2), respectively, cause autosom

29 PKD1 and PKD2 genes, encoding polycystin-1 (PC1) and polycystin-2 (PC2), respectively, lead to autos

30 utations in the genes encoding polycystin-1 (PC1) and polycystin-2 (PC2), which form an ion channel c

31 in PKD1 or PKD2 which encodes polycystin-1 (PC1) and polycystin-2, respectively.

32 The polycystin-1 (PC1) and pVHL proteins may therefore participate in the

33 creens using the C-terminus of polycystin-1 (PC1) as bait.

34 Mutations in polycystin-1 (PC1) give rise to autosomal dominant polycystic kidney d

35 the BBSome, the ADPKD protein polycystin-1 (PC1) interacts with BBS1, BBS4, BBS5 and BBS8, four of t

36 Polycystin-1 (PC1) is a large membrane protein that is expressed along

37 Mutation of polycystin-1 (PC1) is the major cause of autosomal dominant polycystic

38 Mutations in polycystin-1 (PC1) lead to autosomal-dominant polycystic kidney diseas

39 Dysregulation of polycystin-1 (PC1) leads to autosomal dominant polycystic kidney disea

40 Polycystin-1 (PC1) mutations result in proliferative renal cyst growth

41 commonly caused by defects in polycystin-1 (PC1) or polycystin-2 (PC2), in which tubular epithelia f

42 her PKD1 or PKD2, which encode polycystin-1 (PC1) or polycystin-2 (PC2), respectively.

43 KVHPSST, in the C-terminus of polycystin-1 (PC1) that serves as a ciliary-targeting signal.

44 tations in the gene coding for polycystin-1 (PC1) underlie the majority of cases but the function of

45 Mutations in Pkd1, encoding polycystin-1 (PC1), cause autosomal-dominant polycystic kidney disease

46 ns in the gene (PKD1) encoding polycystin-1 (PC1).

47 by mutations in PKD1, encoding polycystin-1 (PC1).

48 idney as the result of reduced polycystin-1 (PC1).

49 utations in the genes encoding polycystin-1 (PC1, PKD1) or polycystin-2 (PC2, PKD2) cause ADPKD, and

50 Through Jade-1, PC1 and PC1 cleaved forms may exert fine control of beta

51 that identification of B-1a.PC1(lo) and B-1a.PC1(hi) cells extends the concept of a layered immune sy

52 se subsets, designated B-1a.PC1(lo) and B-1a.PC1(hi), differ significantly in IgH chain utilization.

53 We conclude that identification of B-1a.PC1(lo) and B-1a.PC1(hi) cells extends the concept of a

54 These subsets, designated B-1a.PC1(lo) and B-1a.PC1(hi), differ significantly in IgH ch

55 Mutations in polycystin 1 and 2 (PC1 and PC2) cause the common genetic kidney disorder au

56 or PKD2 genes, encoding polycystins 1 and 2 (PC1 and PC2).

57 he family of prohormone convertases 1 and 2 (PC1 and PC2).

58 five longest mussel chronologies (1982-2003; PC1(mussel)) accounted for 47% of the dataset variabilit

59 s (PCs) furin and proprotein convertase 1/3 (PC1) cleave substrates at dibasic residues along the euk

60 Proprotein convertase 1/3 (PC1/3) deficiency, an autosomal-recessive disorder cause

61 ene, encoding the proprotein convertase 1/3 (PC1/3), cause recessive monogenic early onset obesity.

62 localization of an integral membrane CD16.7-PC1 chimera.

63 tors Arf4 or Rab8 functionally blocks CD16.7-PC1 trafficking to cilia.

64 By controlling Jade-1 abundance, PC1 and the PC1-CTF differentially regulate Jade-1-media

65 oded by two genes are associated with ADPKD: PC1 (pkd1), primarily a signaling molecule, and PC2 (pkd

66 l determinants of PC activation, we analyzed PC1/3, a paralogue of furin that is activated at a pH of

67 Here we show that membrane-anchored PC1 activates STAT3 in a JAK2-dependent manner, leading

68 Thus, a relationship between Jade-1 and PC1 was sought.

69 Expression of Pdx1, Nkx6.1 and PC1/3 in hESC-derived Ucn 3(+) beta cells supports this.

70 sion models between antioxidant capacity and PC1 and PC2 displayed strong linear correlations for NF

71 Swapping propeptides between furin and PC1 transfers pH-dependent protease activation in a prop

72 PC1(mussel) and PC1(discharge) were closely linked to regional wintertim

73 Through Jade-1, PC1 and PC1 cleaved forms may exert fine control of beta-catenin

74 Further experiments showed that PC1 and PC1-5TMC reduce phosphorylation of eIF2alpha through inh

75 f a common precursor, proglucagon by PC2 and PC1, respectively.

76 he beta-lactam sensor/signal transducer, and PC1 beta-lactamase in four strains of Staphylococcus aur

77 nhibit other proprotein convertases, such as PC1/3, PC4, PACE4, and PC5/6, with similar potency, wher

78 However, the role of large COPII vesicles as PC1 transport carriers was not unambiguously demonstrate

79 ADPKD-associated PC1 mutants failed to regulate Jade-1, indicating a pote

80 INT-777 augmented PC1 expression in alpha cells and stimulated GLP-1 relea

81 g affinity of BLIP for Staphylococcus aureus PC1 beta-lactamase and to identify mutants that alter bi

82 PC1 and PC2 first interact in the ER before PC1 cleavage at the GPS/GAIN site and determined that PC

83 reveal a reciprocal functional link between PC1 and PC2 which is critically dependent on their inter

84 mediated by Siah-1, an E3 ligase that binds PC1.

85 st important variable for explaining biology PC1 variability, and commercial catch the most important

86 and generalized additive models (for biology PC1-2) invoking only the climate modes produced residual

87 idate variables, resulting models of biology PC1-2 satisfied assumptions of independent residuals and

88 ry for taxa strongly associated with biology PC1-2 suggest plausible mechanistic explanations for the

89 sp are in the same protein complex, and both PC1 and Pacsin 2 are required for N-Wasp/Arp2/3-dependen

90 PC2, whose dephosphorylation is mediated by PC1 binding through the recruitment of protein phosphata

91 ndicate that STAT3 signaling is regulated by PC1 and is a driving factor for renal epithelial prolife

92 quitin ligase whose activity is regulated by PC1 in a manner that is physiologic and may correlate wi

93 AMP amplified the activation of Src/STAT3 by PC1-p30.

94 sicles that completely encapsulate the cargo PC1 and are physically separated from ER.

95 Compared with wild-type cells, PC1-depleted immortalized renal collecting duct cells ha

96 membranous COOH-terminal fragment of cleaved PC1 required an intact interaction with PC2.

97 1 to directly activate STAT3 but the cleaved PC1 tail now coactivates STAT3 in a mechanism requiring

98 Both linear models (for climate PC1) and generalized additive models (for biology PC1-2)

99 ariables were the first principle component (PC1) of four regional climate parameters [sea surface te

100 ence EEMs revealed two principal components (PC1-tryptophan, PC2-tyrosine) that captured significant

101 between the first two principle components (PC1-PC2) best represented ARHI.

102 PC1, compared with control cells containing PC1.

103 In contrast, PC1(hi) cells produced more IL-10 than PC1(lo) cells whe

104 elix 2 (NHLH2) and the prohormone convertase PC1 (encoded by PCSK1) were reduced in PWS patient induc

105 The prohormone convertases PC1/3 and PC2 are eukaryotic serine proteases involved i

106 es showed that catecholamines inhibited CPE, PC1/3, and PC2, with dopamine quinone the most potent in

107 In proximal tubule-derived, PC1-knock-out cells, adenylyl cyclase 6 and 3 (AC6 and -

108 ding principal component of river discharge (PC1(discharge); r = -0.88; P < 0.0001).

109 type 1 (Pcsk1) expression, the gene encoding PC1/3, which controls GLP-1 production, was decreased in

110 creased the surface expression of endogenous PC1 and PC2 in vitro and in vivo and increased Wnt-activ

111 est that a test measuring the urine exosomal PC1/TMEM2 or PC2/TMEM2 ratio may have utility in diagnos

112 deformation associated with canal expansion (PC1), anterior-posterior deformation of the LC and the f

113 In pulldown experiments, PC1 bound to Galpha(12), but not the related Galpha(13)

114 cells elaborated primary cilia and expressed PC1, PC2, and FPC at similar levels, and PKD and control

115 s prepared from PC12 cells stably expressing PC1/3 or PC2.

116 Genome-wide association analyses for PC1-3 were conducted separately in each sample assuming

117 that PC2 acts as an essential chaperone for PC1 maturation and surface localization.

118 The BLIP inhibition constant (K(i)) for PC1 beta-lactamase was measured at 350 nM, and isotherma

119 determined that GPS cleavage is required for PC1 trafficking to cilia.

120 mpelling results support a critical role for PC1 deficiency in PWS, more work needs to be done to ful

121 es ranged from 0.70 (95% CI: 0.63-0.76) for (PC1-PC2) to 0.56 (95% CI: 0.48-0.63) for PC2.

122 Peritoneal macrophages isolated from PC1/3 KO mice also demonstrate elevated cytokine secreti

123 at animals with reduced levels of functional PC1 and PC2 in the kidney exhibited severe, rapidly prog

124 rylation of PC2 in the absence of functional PC1 could contribute to cyst initiation in PKD1 patients

125 s important role in neuroendocrine functions PC1/3 also has an important role in the regulation of th

126 Furthermore, PC1(lo) cells generated antigen-specific IgM responses t

127 fic antibodies have been raised using hapten PC1 (a 1:1 mixture of 9-hydroxy- and 6-hydroxy-phenazine

128 Hence, PC1 can regulate STAT activity by a dual mechanism.

129 However, PC1/3 is also expressed in cells of the immune system, m

130 protein EGLN3 (or PHD3), which hydroxylates PC1.

131 f large cargo, such as 300-nm procollagen I (PC1) molecules, from the endoplasmic reticulum (ER) to t

132 e libraries for tight binders to immobilized PC1 beta-lactamase.

133 requent coding variants that modestly impair PC1/3 function mildly increase the risk for common obesi

134 emonstrate a depletion of dendritic cells in PC1/3 KO spleens.

135 of PKD cell lines, which exhibit defects in PC1 expression and collagen compaction.

136 ogenesis is most likely driven by defects in PC1 maturation.

137 Humans and mice deficient in PC1 display hyperphagic obesity, hypogonadism, decreased

138 F-alpha) were very significantly elevated in PC1/3 KO mice, consistent with a hypercytokinemia, i.e.

139 n-coupled receptor proteolysis site (GPS) in PC1.

140 l inhibition of HDAC6 reduced cyst growth in PC1-knock-out mice.

141 Intracellular Ca(2+) was higher in PC1-knock-out cells than in control cells.

142 (acetoxymethyl ester) reduced cAMP levels in PC1-knock-out cells.

143 ta cell stimulus-secretion pathway including PC1/3, PC2, GLUT-1, glucokinase, and K-ATP channel compl

144 c alpha cells enhances hyperglycemia-induced PC1 expression thereby releasing GLP-1, which in turn in

145 Interestingly, PC1 does not activate autophagy generally.

146 unds that were able to stimulate both 87 kDa PC1/3 and PC2 activity, behavior related to the presence

147 In ADPKD kidneys PC1 tail fragments are overexpressed, including a unique

148 Moreover, cells lacking PC1 expression use less O2 and show less mitochondrial C

149 otein expression was higher in cells lacking PC1, compared with control cells containing PC1.

150 phosphorylated in cells and tissues lacking PC1.

151 ds to PC2 and that expression of full-length PC1 accelerates the transport of the HDAC6-PC2 complex t

152 Full-length PC1 appeared as two unequally sized blobs connected by a

153 Full-length PC1 bound, stabilized and colocalized with Jade-1 and in

154 We show here that full-length PC1 that interacts with PC2 via a C-terminal coiled-coil

155 When challenged with lipopolysaccharide, PC1/3 KO mice are more susceptible to septic shock than

156 In Caenorhabditis elegans and mammals, PC1 and PC2 act in the same genetic pathway, act in a se

157 D proteins (PC1 and PC2), and reduced mature PC1 was seen in GANAB(+/-) cells.

158 e is associated with the level of the mature PC1 glycoform.

159 nequimolar reduction (20%-25%) of the mature PC1 glycoform.

160 regulates PC1 maturation; therefore, mature PC1 levels are a determinant of disease severity in PKD2

161 nvertase (PC) family comprises nine members: PC1/3, PC2, furin, PC4, PC5/6, PACE4, PC7, SKI-1/S1P, an

162 A novel PC1/3 variant introducing an Arg to Gln amino acid subst

163 o the presence of alanine at position 104 of PC1.

164 Tail-cleavage abolishes the ability of PC1 to directly activate STAT3 but the cleaved PC1 tail

165 ma-secretase activity impairs the ability of PC1 to suppress growth and apoptosis and leads to cyst f

166 In the absence of PC1-p30, cAMP dampened EGFR- or IL-6-dependent activatio

167 ment-binding protein-dependent activation of PC1.

168 the maturation or the enzymatic activity of PC1/3 in cell lines.

169 aturation and in vitro catalytic activity of PC1/3.

170 ase in the setting of impaired biogenesis of PC1.

171 g reaction that reconstitutes the capture of PC1 into large COPII vesicles.

172 rge COPII vesicles are bona fide carriers of PC1.

173 pliced XBP1 (XBP1s) enhanced GPS cleavage of PC1 in SEC63-deficient cells, and XBP1 overexpression in

174 The genetic correlation of PC1-PC2 and SNR was -0.67 showing that the 2 traits shar

175 show that the carboxy-terminal tail (CTT) of PC1 is released by gamma-secretase-mediated cleavage and

176 , and alpha-toxin (PLAT) signature domain of PC1 using nuclear magnetic resonance, biochemical, cellu

177 Expression of PC1-p30 changed the cellular response to cAMP signaling.

178 missense mutations is a resulting failure of PC1 to traffic to cilia regardless of GPS cleavage.

179 estigated clinical and molecular features of PC1/3 deficiency.

180 and confirmed that only the cleaved form of PC1 exits the ER and can rescue the embryonically lethal

181 e naturally occurring C-terminal fragment of PC1 (PC1-CTF) promoted Jade-1 ubiquitination and degrada

182 A proteolytic fragment of PC1 corresponding to the cytoplasmic tail, PC1-p30, is o

183 lates the surface expression and function of PC1 and PC2.

184 ie the majority of cases but the function of PC1 has remained poorly understood.

185 pite its medical importance, the function of PC1 remains poorly understood.

186 in PC7 KO mice but not in the hippocampus of PC1/3 KO mice.

187 orm leads to stunted cilia and inhibition of PC1 on primary cilia.

188 phosphorylation-dependent internalization of PC1 is closely linked to its function in renal developme

189 Involvement of PC1-regulated eIF2alpha phosphorylation and a PKR-eIF2al

190 m individuals with PKD1 mutations, levels of PC1 and PC2 were reduced to 54% (P<0.02) and 53% (P<0.00

191 controlling the subcellular localization of PC1 and PC2 are poorly understood.

192 Loss of PC1 causes increased proliferation and apoptosis, while

193 Loss of PC1 expression profoundly alters cellular energy metabol

194 required for GPS cleavage and maturation of PC1, and activation of XBP1 can protect against polycyst

195 ein expression levels, and overexpression of PC1 but not a carboxy-terminal truncation mutant increas

196 dent activation of STAT3; in the presence of PC1-p30, cAMP amplified Src-dependent activation of STAT

197 various histidines within the propeptide of PC1/3 and examined how such alterations can modulate pH-

198 me in the discovery cohort, and the ratio of PC1/TMEM2 or PC2/TMEM2 could be used to distinguish indi

199 in diameter and accelerates the secretion of PC1.

200 soleucine and valine on the negative side of PC1 and porcine gelatin was correlated to the polar side

201 erine and methionine on the positive side of PC1; bovine gelatin was correlated to the non-polar side

202 roteins involved in the endosomal sorting of PC1 and PC2 could lead to new therapeutic approaches in

203 rst direct visualization of the structure of PC1, and reveal the architecture of the protein, with in

204 The C-terminal cytoplasmic tail of PC1 can undergo proteolytic cleavage and nuclear translo

205 A key target of PC1, the cyclin-dependent kinase inhibitor p21, is also

206 length nor the plasma membrane targeting of PC1.

207 Deletion of the extreme C-terminus of PC1 ablates Arf4 and ASAP1 binding and prevents ciliary

208 e mechanisms that control the trafficking of PC1 and PC2, as well as their broader physiological role

209 kout of BBS4, impairs ciliary trafficking of PC1 in kidney epithelial cells.

210 i.e. 13-fatty, falling at positive value of PC1; this fit the aroma perception of this varietal.

211 of Negroamaro wines have negative values of PC1 and they are negatively correlated with the second m

212 04) variant both exhibits adverse effects on PC1/3 activity and is prevalent in the population sugges

213 btilisin/kexin type 1 with modest effects on PC1/3 in vitro have been associated with obesity in five

214 d alpha cells do not express Nkx6.1, Pdx1 or PC1/3 in agreement with the presence of a separate popul

215 urally occurring C-terminal fragment of PC1 (PC1-CTF) promoted Jade-1 ubiquitination and degradation,

216 conserved in the PC1 propeptide (PRO(PC1)), PC1 nonetheless activates at pH~5.5 within the dense cor

217 that designates these as the CG2, GC2, PC3, PC1, C3, and GP2 classes of superbases.

218 E (CPE) and the prohormone convertases (PCs) PC1/3 and PC2.

219 riments to determine whether the polycystins PC1 and PC2 (encoded by Pkd1 and Pkd2) and the transcrip

220 Mutations in polycystins (PC1 or PC2/TRPP2) cause progressive polycystic liver dis

221 Mutations that prevent PC1's GPS cleavage prevent its plasma membrane localizat

222 Pkd2-/- mice, complete loss of PC2 prevented PC1 maturation.

223 s establish that while both PRO(FUR) and PRO(PC1) are enriched in histidines when compared with cogna

224 We further demonstrate that PRO(FUR) and PRO(PC1) are sufficient to confer organelle sensing on foldi

225 activation and examine why PRO(FUR) and PRO(PC1) differ in their pH-dependent activation.

226 PRO(FUR) is ~2-fold greater than that in PRO(PC1), which may augment its pH sensitivity.

227 idue is conserved in the PC1 propeptide (PRO(PC1)), PC1 nonetheless activates at pH~5.5 within the de

228 cantly unfolds PRO(FUR) when compared to PRO(PC1) to enhance autoproteolysis.

229 We identified four promising PC1/3 competitive inhibitors and three PC2 inhibitors th

230 We propose that the 11-span membrane protein PC1 is a BBSome cargo and that the components of the BBS

231 ciliary localization of the ADPKD proteins (PC1 and PC2), and reduced mature PC1 was seen in GANAB(+

232 e measured enzymatic activity of recombinant PC1/3 proteins.

233 ystic disease in a murine model with reduced PC1 function that is unrelated to SEC63 inactivation.

234 Our results indicate that PC2 regulates PC1 maturation; therefore, mature PC1 levels are a deter

235 iferation and apoptosis, while reintroducing PC1-CTT into cultured Pkd1 null cells reestablishes norm

236 Because of its size, PC1 has proven difficult to handle biochemically, and st

237 was found to both inhibit PC2 and stimulate PC1/3.

238 ductions in islet, hypothalamic, and stomach PC1 content.

239 f PC1 corresponding to the cytoplasmic tail, PC1-p30, is overexpressed in ADPKD.

240 We found that the PLAT domain targets PC1 to the plasma membrane in polarized epithelial cells

241 ranslocation of the PC1 C-terminal tail/TAZ (PC1-CTT/TAZ) complex, leading to increased runt-related

242 e segments and the intracellular C-terminus (PC1-5TMC) down-regulate the phosphorylation of protein k

243 rast, PC1(hi) cells produced more IL-10 than PC1(lo) cells when stimulated with LPS and phorbol 12-my

244 PC1(hi) cells were also more efficient than PC1(lo) cells in regulating Th1 cell differentiation, ev

245 culating natural IgM and intestinal IgA than PC1(hi) cells.

246 knockout mouse kidney epithelial cells that PC1 and its truncation mutant comprising the last five t

247 The results of this study demonstrate that PC1 trafficking and expression require GPS cleavage and

248 We further demonstrate that PC1, Pacsin 2 and N-Wasp are in the same protein complex

249 Here, we demonstrated that PC1 and PC2 first interact in the ER before PC1 cleavage

250 eIF2alpha phosphorylation, demonstrated that PC1-5TMC inhibits apoptosis of HEK293T cells in a PKR-eI

251 Here we describe that PC1 and PC2 must interact and form a complex to reach th

252 mmunoprecipitation experiments we found that PC1 truncation mutants associate with PKR, or with PKR a

253 We found that PC1(lo) cells develop from an early wave of B-1a progeni

254 These findings indicate that PC1/3 is involved in the processing of one or more enter

255 n-induced ER Ca(2+) release, indicating that PC1 can modulate mitochondrial function.

256 We propose that PC1 modulates actin cytoskeleton rearrangements and dire

257 Here, we report that PC1 interacts with Pacsin 2, a cytoplasmic phosphoprotei

258 Here, we show that PC1-p30 interacts with the nonreceptor tyrosine kinase S

259 Further experiments showed that PC1 and PC1-5TMC reduce phosphorylation of eIF2alpha thr

260 We have now shown that PC1 overexpression leads to increased degradation of PC2

261 We have previously shown that PC1 regulates the transcriptional activity of signal tra

262 Our results suggest that PC1 acts as a negative regulator of STAT3 and that block

263 Taken together, these results suggest that PC1 regulates ciliary PC2 protein expression levels and

264 These results suggest that PC1, via its cleaved cytoplasmic tail, integrates signal

265 own-regulate PC2 expression, suggesting that PC1-PC2 interaction is necessary for PC2 regulation.

266 The PC1-p30-mediated activation of Src/STAT3 was independent

267 The PC1/3 knock-out (KO) mouse model has allowed us to eluci

268 The PC1/TMEM2 ratio correlated inversely with height-adjuste

269 By controlling Jade-1 abundance, PC1 and the PC1-CTF differentially regulate Jade-1-mediated transcri

270 results reveal a physiological role for the PC1-PLAT domain in renal epithelial cells and suggest th

271 Although this residue is conserved in the PC1 propeptide (PRO(PC1)), PC1 nonetheless activates at

272 nflammatory T(h)1 pathway is dominant in the PC1/3 KO mouse model.

273 he effects of innate immune challenge in the PC1/3 KO mouse.

274 d a compound predicted to bind to PC2 in the PC1:PC2 C-terminal tail region with helix:helix interact

275 ne selected, and it was found to inhibit the PC1 beta-lactamase with a K(i) of 42 nM, while calorimet

276 stimulated the nuclear translocation of the PC1 C-terminal tail/TAZ (PC1-CTT/TAZ) complex, leading t

277 us, gamma-secretase-dependent release of the PC1-CTT creates a protein fragment whose expression is s

278 Expression of the PC1-CTT is sufficient to rescue the dorsal body curvatur

279 ecular modeling revealed interactions of the PC1/3 inhibitors with the active site that suggest struc

280 eper" that fine-tunes the sensitivity of the PC1/3 propeptide to facilitate the release inhibition at

281 Proline substitutions on the PC1-proximal side completely abolished transport and red

282 hypothesis was confirmed by showing that the PC1(A104E) enzyme binds BLIP with 15-fold greater affini

283 dies suggested that BLIP binds weakly to the PC1 beta-lactamase due to the presence of alanine at pos

284 increased interaction of Galpha(12) with the PC1 C terminus.

285 n PC1 is expressed, PC2 that is not bound to PC1 is directed to aggresomes and subsequently degraded

286 or neuroendocrine features of PWS are due to PC1 deficiency.

287 ine concentrations found to be inhibitory to PC1/3, PC2, and CPE are well within the physiological ra

288 Adoptively transferred PC1(lo) cells secreted significantly more circulating na

289 tins and the clathrin adaptor AP2 to trigger PC1 internalization.

290 with 15-fold greater affinity than wild-type PC1 beta-lactamase.

291 Furthermore, ectopic expression of wild-type PC1 in ADPKD iPS-derived hepatoblasts rescued ciliary PC

292 Here we have isolated wild-type PC1, and several mutants, from transfected cells by immu

293 The structures of the various PC1 mutants were all consistent with this model.

294 uncovered a new pathway suggesting that when PC1 is expressed, PC2 that is not bound to PC1 is direct

295 neumococcal polysaccharide antigens, whereas PC1(hi) cells do not.

296 e of B-1a progenitors in fetal life, whereas PC1(hi) cells are generated from a later wave after birt

297 (ELVs) (100-nm diameter vesicles), in which PC1 is present in a cleaved form and may be complexed wi

298 ed the clinical features of 13 children with PC1/3 deficiency and performed sequence analysis of PCSK

299 In a study of 13 children with PC1/3 deficiency caused by disruption of PCSK1, failure

300 UPR and that SEC63 exists in a complex with PC1.