ライフサイエンスコーパス: NCL

1 NCL also regulates the biogenesis of specific microRNAs

2 NCL is therefore suited to participate in a neural circu

3 NCL knockdown specifically reduced the ORF2 IRES activit

4 NCL overexpression increased EBNA1 binding to oriP and t

5 NCL receives telencephalic projections from the hyperstr

6 NCL repression of p53 translation utilizes both the 5'-

7 NCL RNA-binding domain K429 was critical for ATP and EBN

8 PL26, respectively, and that disruption of a NCL-NCL homodimer by RPL26 may be the switch between tra

9 us hydrazine to cleave the Ddae linker after NCL-based assembly.

10 ction can be disrupted by the addition of an NCL binding aptamer (AS1411).

11 EBNA1's N-terminal 100 aa and NCL's RNA-binding domains were critical for EBNA1/NCL in

12 A, is required for both NCL dimerization and NCL-mediated translational repression, and is the domain

13 to loss of PGRN contributes to both FTD and NCL pathology in a dose-dependent manner.

14 hilic deposits, suggesting that both FTD and NCL-like pathology are present in PGRN patient neurons a

15 es both the 5'- and 3'-UTRs of p53 mRNA, and NCL binds to the same 5'-3'-UTR interaction region that

16 tical interaction between DENV C protein and NCL that represents a potential new target for the devel

17 We confirmed reports that RPL26 and NCL interact with each other and then explored the poten

18 e describe, to our knowledge, the first anti-NCL single-chain fragment variable displaying antineopla

19 novel therapeutic target for GRN-associated NCL and FTD and highlight the emerging theme of defectiv

20 erts to arylthioesters that are efficient at NCL.

21 epresent the prototype of novel immune-based NCL-targeting drugs with clinical potential as diagnosti

22 sent work was to study the interplay between NCL and alpha(v)beta(3) by using biochemical, immunofluo

23 projection also suggests similarity between NCL and mammalian frontal cortical areas.

24 This immunoagent binds NCL on the cell surface, it is translocated into the cyt

25 in binding to p53 mRNA, is required for both NCL dimerization and NCL-mediated translational repressi

26 al repression and stress induction of p53 by NCL and RPL26, respectively, and that disruption of a NC

27 ation area termed nidopallium caudolaterale (NCL) from crows that assessed and briefly memorized nume

28 elencephalic area nidopallium caudolaterale (NCL) while two crows (Corvus corone) performed a delayed

29 le neurons in the nidopallium caudolaterale (NCL), a pallial association area of the avian endbrain.

30 rons in the avian nidopallium caudolaterale (NCL), an endbrain structure that originated independentl

31 e-spanning proteins, whose deficiency causes NCL in mouse and man.

32 al ligation and thiol-ene radical chemistry (NCL-TEC) on the resulting cysteine thiol has been invest

33 rompted the isolation of several sub-clones (NCL-1-12) to identify a more tractable and improved in v

34 vivo than its noncleavable (NC) analog, CMP-NCL-CA4.

35 t stem cells (iPSCs) for the two most common NCL subtypes: classic late-infantile NCL, caused by TPP1

36 hich the infantile NCL (INCL) and congenital NCL (CNCL) are the most lethal.

37 disease), which is second only to congenital NCL for its age of onset and devastating progression.

38 CLN3 is the most widely conserved NCL gene, suggesting that it has a basic eukaryotic cell

39 d working memory of numerosities in the crow NCL exhibiting several characteristics that are surprisi

40 mmunolabelling with the antibody (designated NCL-hamlet) demonstrated a polypeptide of approximately

41 ronal ceroid lipofuscinosces/Batten disease (NCL) is a devastating group of neurodegenerative disease

42 Excessive RPL26 disrupts NCL dimerization, and point mutations in the NCL-interac

43 Dorsal NCL contained relatively dense staining for TH, choline

44 d for investigating early mechanisms driving NCL pathogenesis.

45 RNA-binding domains were critical for EBNA1/NCL interaction.

46 tly inhibited nucleolin expression, enhanced NCL mRNA association with argonaute-containing complexes

47 ected against the full-length human ERalpha (NCL-ER-6F11), calcium-binding proteins calbindin-D(28k),

48 was accomplished through a menu of extended NCL followed by metal free dethiylation.

49 e linker can be cleaved in one pot following NCL or desulfurization.

50 PSAP is an underlying disease mechanism for NCL and FTLD due to GRN mutations.

51 points postinfection, suggesting a role for NCL in viral morphogenesis.

52 ents reveal a surprisingly critical role for NCL K429 in EBNA1 episome maintenance and transcription,

53 motif common to CL-extensins but absent from NCL-extensins was Val-Tyr-Lys.

54 In NCL, lysosomes accumulate autofluorescent proteolipid in

55 ed with the survival of patients with HCC in NCL although the therapeutic options differ from those f

56 Data on patients with HCC in NCL in advanced stages are scarce.

57 Only a minority of patients with HCC in NCL lacked any sign of hepatic damage.

58 More than 25% of patients with HCC in NCL presented with extrahepatic metastases.

59 performed comparing 93 patients with HCC in NCL to 571 patients with HCC in liver cirrhosis (LC) wit

60 influencing survival in patients with HCC in NCL were analyzed.

61 Patients with HCC in NCL were diagnosed at older age and in more advanced tum

62 sent in the majority of patients with HCC in NCL.

63 nal peptide-thioesters, key intermediates in NCL.

64 ffects that are similar to those observed in NCL.

65 We contend that NMD targets PTCs in NCL gene transcripts for degradation.

66 widespread pathology similar to that seen in NCL.

67 alpha-thioesters that were directly used in NCL without purification.

68 argonaute-containing complexes, and induced NCL RNA transport to PBs.

69 Infantile NCL (INCL), the most severe form of NCL, is caused by mu

70 These are infantile NCL (INCL; CLN1), classical late infantile NCL (LINCL; C

71 of all disease causing alleles in infantile NCL, the most common of which worldwide is the p.R151X m

72 most severe form of the disease is infantile NCL (INCL).

73 Several atypical forms of late infantile NCL (LINCL) have also been described including a Finnish

74 e NCL (INCL; CLN1), classical late infantile NCL (LINCL; CLN2) and juvenile NCL (JNCL; CLN3).

75 to that which occurs in human late infantile NCL.

76 The gene for variant late-infantile NCL (vLINCL), CLN6, was previously mapped to chromosome

77 common NCL subtypes: classic late-infantile NCL, caused by TPP1(CLN2) mutation, and juvenile NCL, ca

78 n an ovine model of a variant late-infantile NCL, there is abnormal expression of sleep homeostasis.

79 d Cln6(nclf) model of variant late-infantile NCL.

80 ultrastructural characteristics of infantile NCL (INCL): granular osmiophilic deposits (GROD).

81 models of NCL: Ppt1(-/-) model of infantile NCL and Cln6(nclf) model of variant late-infantile NCL.

82 rred with a suspected diagnosis of infantile NCL who had normal PPT activity.

83 arious types of NCLs, of which the infantile NCL (INCL) and congenital NCL (CNCL) are the most lethal

84 The infantile NCL (INCL) is the most devastating neurodegenerative LSD

85 ns in CLN1 primarily manifest with infantile NCL (INCL or Haltia-Santavuori disease), which is second

86 missense mutations associated with infantile NCL showed no residual enzyme activity, whereas mutation

87 lity of two novel KDM1A-specific inhibitors (NCL-1 and NCD-38) to promote differentiation and apoptos

88 Interestingly, NCL is expressed on the surface of actively proliferatin

89 ine cerebellar cells derived from a juvenile NCL model (CLN3) showed enrichment of endogenous KCTD7.

90 ate infantile NCL (LINCL; CLN2) and juvenile NCL (JNCL; CLN3).

91 caused by TPP1(CLN2) mutation, and juvenile NCL, caused by CLN3 mutation.

92 nical history is characteristic for juvenile NCL.

93 l mutant (Cln3(-/-)) mouse model of juvenile NCL (JNCL) that revealed a delayed onset neurodegenerati

94 er, mutations in CLN3 are linked to juvenile NCL (JNCL), the latest onset and mildest form of NCL in

95 s a clinical course consistent with juvenile NCL (JNCL) but the ultrastructural characteristics of in

96 patients originally diagnosed with juvenile NCL.

97 en ruled out for mutations in the nine known NCL-associated genes, suggesting that additional genes r

98 The NEXUS Class Library (NCL) is a collection of C++ classes designed to simplify

99 Native chemical ligation (NCL) affords an N-linked chitobiose glycoprotein analogu

100 Although native chemical ligation (NCL) and related chemoselective ligation approaches prov

101 of the machine via native chemical ligation (NCL) demonstrates that even challenging 15- and 19-membe

102 e broad utility of native chemical ligation (NCL) in protein synthesis has fostered a search for meth

103 Native chemical ligation (NCL) is widely applicable for building proteins in the l

104 as performed using native chemical ligation (NCL) of a C-terminal peptide thioester and an N-terminal

105 n order to perform native chemical ligation (NCL) of two peptide fragments.

106 obes combining the native chemical ligation (NCL) reaction with solid phase peptide synthesis (SPPS).

107 sn(36) by means of native chemical ligation (NCL) to give the full sequence of diptericin epsilon.

108 escribe the use of native chemical ligation (NCL) to rapidly prepare phospholipids spontaneously from

109 ive and reversible native chemical ligation (NCL) which can take place at N-(methyl)-cysteine residue

110 In one approach, native chemical ligation (NCL), short, unprotected peptides are connected through

111 eptide by means of native chemical ligation (NCL).

112 ue was achieved by native chemical ligation (NCL).

113 predicted to contain a non-conserved linker (NCL) sequence flanked by highly conserved N- and C-termi

114 n disease or neuronal ceroid lipofuscinoses (NCL) are a group of genetic neurodegenerative diseases t

115 The neuronal ceroid lipofuscinoses (NCL) are progressive neurodegenerative disorders with on

116 Neuronal ceroid-lipofuscinoses (NCL) are autosomal recessive disorders that form the mos

117 The neuronal ceroid lipofuscinoses (NCLs) are a group of autosomal recessive neurodegenerati

118 he childhood neuronal ceroid lipofuscinoses (NCLs) are a group of autosomal recessive neurodegenerati

119 Human neuronal ceroid lipofuscinoses (NCLs) are a group of genetic neurodegenerative diseases

120 The neuronal ceroid lipofuscinoses (NCLs) are a group of inherited and incurable neurodegene

121 The neuronal ceroid lipofuscinoses (NCLs) are common neurodegenerative disorders of childhoo

122 Neuronal ceroid lipofuscinoses (NCLs) are neurodegenerative storage diseases characteriz

123 Neuronal ceroid lipofuscinoses (NCLs) are the most common childhood neurodegenerative LS

124 Neuronal ceroid lipofuscinoses (NCLs) are the most common hereditary neurodegenerative d

125 Neuronal ceroid lipofuscinoses (NCLs) comprise a heterogeneous group of metabolic storag

126 The neuronal ceroid lipofuscinoses (NCLs), also known as Batten disease, are a group of auto

127 Neuronal ceroid lipofuscinoses (NCLs), as a group, represent one of the most common (one

128 ncluding the neuronal ceroid lipofuscinoses (NCLs).

129 Neuronal ceroid lipofuscinoses (NCLs; Batten disease) are collectively the most frequent

130 Neuronal ceroid lipofuscinosis (NCL) comprises approximately 13 genetically distinct lys

131 atients with neuronal ceroid lipofuscinosis (NCL) develop neurodegeneration characterized by vision l

132 se models of neuronal ceroid lipofuscinosis (NCL) exhibit many features of the human disorder, with w

133 Neuronal ceroid lipofuscinosis (NCL) is a genetically heterogeneous group of lysosomal d

134 te infantile neuronal ceroid lipofuscinosis (NCL) is an inherited disorder characterized by progressi

135 A subtype of neuronal ceroid lipofuscinosis (NCL) is well recognized which has a clinical course cons

136 ses known as neuronal ceroid lipofuscinosis (NCL) or Batten disease has been identified.

137 h variant of neuronal ceroid lipofuscinosis (NCL) or Batten disease, due to defects in a putative new

138 model human neuronal ceroid lipofuscinosis (NCL) or Batten disease.

139 tive disease neuronal ceroid lipofuscinosis (NCL), accumulated throughout the CNS but not in visceral

140 Neuronal ceroid lipofuscinosis (NCL), commonly referred to as Batten disease, is a group

141 me (KRS) and neuronal ceroid lipofuscinosis (NCL).

142 ge disorder, neuronal ceroid lipofuscinosis (NCL).

143 n, infantile neuronal ceroid lipofuscinosis (NCL).

144 r, infantile neuronal ceroid lipofuscinosis (NCL).

145 age disorder neuronal ceroid lipofuscinosis (NCL).

146 clusions and neuronal ceroid lipofuscinosis (NCL).

147 ine model of neuronal ceroid lipofuscinosis (NCL, Batten disease) caused by a mutation in CLN5 In hum

148 a family of neuronal ceroid lipofuscinosis (NCLs) diseases.

149 hy (SMA) and neuronal ceroid lipofuscinosis (NCLs).

150 HCC is diagnosed in the non-cirrhotic liver (NCL).

151 We show that many NCL neurons encode information about visual stimuli and

152 Lentivirus shRNA-mediated NCL depletion substantially reduced EBNA1 recruitment to

153 orization of numerosities in working memory, NCL activity predicted the crows' number discrimination

154 preclinical studies using orthotopic models, NCL-1 and NCD-38 significantly reduced GSCs-driven tumor

155 Moreover, NCL silencing impaired lymphoblastoid cell line growth.

156 was rescued by reintroducing the non-mutated NCL proteins.

157 The pigeon caudolateral neostriatum (NCL) shares a dopaminergic innervation with mammalian fr

158 the homing pigeon caudolateral neostriatum (NCL).

159 his process, suppressor-26 (sup-26) and NHL (NCL-1, HT2A, and LIN-41 repeat) domain-containing-2 (nhl

160 me) but not in the caudolateral nidopallium (NCL) or hippocampus.

161 Nucleolin (NCL) is a nucleocytoplasmic protein involved in many bio

162 Nucleolin (NCL) was identified to be EBNA1 associated.

163 RPL4 and Nucleolin (NCL) were a scaffold for an EBNA1-induced oriP complex.

164 proteins (hnRNPs) R, Q and L, and nucleolin (NCL), appeared to interact specifically with the ORF2 IR

165 In contrast, nucleolin (NCL) suppresses the translation of p53 mRNA and its indu

166 The multifunctional protein nucleolin (NCL) is overexpressed on the surface of activated endoth

167 the multifunctional host protein nucleolin (NCL).

168 Here we find that nucleolin (NCL), a major nucleolar protein, posttranscriptionally r

169 uR was found to interact with the nucleolin (NCL) 3'UTR and specifically promoted nucleolin translati

170 This suggests that sustained activity of NCL neurons is a neuronal correlate of visual working me

171 This work expands the application of NCL to the formation of phospholipid membranes.

172 et cells, and it abrogates the biogenesis of NCL-dependent miRNAs.

173 ion of lipofuscin deposits characteristic of NCL, thus providing the first direct demonstration that

174 Forebrain connections of NCL show that somatosensory, visual, and olfactory infor

175 We found that the RNA-binding domain of NCL participates in binding to p53 mRNA, is required for

176 anslational repression, and is the domain of NCL that interacts with RPL26.

177 affected by a molecularly undefined form of NCL characterized by infantile-onset progressive myoclon

178 (JNCL), the latest onset and mildest form of NCL in children.

179 nfantile NCL (INCL), the most severe form of NCL, is caused by mutations in the Ppt1 gene, which enco

180 Cln3 (-/-) mouse model of a juvenile form of NCL.

181 gliosis is a characteristic of all forms of NCL, but it is unclear whether glial activation precedes

182 of the molecularly uncharacterized forms of NCL, we report here the structure and chromosomal locali

183 associated phenotypes common to all forms of NCL.

184 s were identified, suggesting other forms of NCL.

185 Finally, inhibition of NCL using guanosine-rich aptamers reduces the levels of

186 Knockdown of NCL with small interfering RNA (siRNA) or treatment of c

187 Localization studies show that the level of NCL-1 protein is independently regulated in different ce

188 he largest nucleoli have the lowest level of NCL-1 protein.

189 uanosine-rich aptamers reduces the levels of NCL-dependent miRNAs and their target genes, thus reduci

190 ) determine the cell surface localization of NCL downstream of the RPTPbeta/zeta/c-Src signaling casc

191 tein is feasible using the combined logic of NCL and metal-free dethiylation (MFD).

192 x-dependent apoptosis in this mouse model of NCL, combined Bax- and CD-deficient mice were generated.

193 olvement of interneurons in a mouse model of NCL.

194 the brains of two different mouse models of NCL: Ppt1(-/-) model of infantile NCL and Cln6(nclf) mod

195 ast two neurochemically distinct portions of NCL.

196 ide was more abundant in ventral portions of NCL.

197 nd to be densest in intermediate portions of NCL.

198 ) play in the instigation and progression of NCL pathogenesis.

199 and cell-cycle regulation in both strains of NCL mice.

200 ve have been reported recently in subsets of NCL patients.

201 that its expression correlates with that of NCL-dependent miRNAs.

202 dentified as the cause of different types of NCL, with ages at onset ranging from around birth to adu

203 ing to five overlapping clinical variants of NCL.

204 e first clinical signs in childhood forms of NCLs.

205 tion reported in clinical childhood forms of NCLs.

206 enes (called CLNs) underlie various types of NCLs, of which the infantile NCL (INCL) and congenital N

207 ath to novel therapeutic approaches based on NCL-targeting aptamers for the modulation of miRNA expre

208 nucleolar protein fibrillarin, dependent on NCL-1.

209 ent study of a sibling pair with adult onset NCL and retinal degeneration showed linkage to the regio

210 this gene may be a candidate for adult-onset NCL.

211 mutations also cause a rare, infantile-onset NCL subtype designated as CLN14.

212 isease-modifying therapies for either FTD or NCL, in part because of a poor understanding of how muta

213 ing or CL-extensins and non-cross-linking or NCL-extensins.

214 Transfection with CLN8 but not other NCL genes corrected growth and apoptosis in CLN9-deficie

215 nd sequencing of the coding regions of other NCL genes was negative.

216 vides a possible explanation for overlapping NCL-like pathology observed in patients with mutations i

217 r regression, premature death, and prominent NCL-type storage material.

218 in the nclf mouse, which manifests recessive NCL-like disease, disclosed a third lesion-an extra base

219 n the NCL-interacting region of RPL26 reduce NCL-RPL26 interactions and attenuate both RPL26 binding

220 s were developed that allowed the sequential NCL-TEC process to proceed in high yield.

221 uronal populations in the mnd/mnd mouse show NCL-like pathological changes.

222 The ligation strategies (NCL, Staudinger, KAHA, KATs, etc.) that could involve bo

223 yielding thioester peptides and subsequently NCL reaction.

224 Positive correlation of cell surface NCL and alpha(v)beta(3) expression was also observed in

225 d as a biomarker for the use of cell surface NCL antagonists as anticancer agents.

226 inhibition of cell migration by cell surface NCL antagonists was observed only in cells expressing al

227 Interestingly, cell surface NCL localization was detected only in cells expressing a

228 ty mediated this phenomenon and cell surface NCL was found to interact with both alpha(v)beta(3) and

229 Immunoprecipitation of MS2-tagged NCL 3'UTR was used to screen for endogenous repressors o

230 We also found that NCL is able to oligomerize, consistent with a model in w

231 3'UTR bearing MS2 RNA hairpins revealed that NCL RNA was mobilized to processing bodies (PBs) after s

232 Moreover, the present study reveals that NCL is reached by a limbic projection from the nucleus t

233 These data show that NCL and KRS may share etiological features and implicate

234 We also show that NCL is commonly overexpressed in human breast tumors and

235 Here we show that NCL-1/TRIM2/Brat tumour suppressor extends lifespan and

236 The results suggest that NCL may consist of multiple subdivisions.

237 The NCL is freely available under the GNU General Public Lic

238 droxylase (TH)-dense region that defines the NCL.

239 ver, protein sequencing failed to detect the NCL.

240 lementary information: Documentation for the NCL (general information and source code documentation)

241 We recorded single-neuron activity from the NCL of crows performing a delayed match-to-sample task w

242 Thus, sustained activity in the NCL actively processes information for the upcoming beha

243 NCL dimerization, and point mutations in the NCL-interacting region of RPL26 reduce NCL-RPL26 interac

244 ermore, while recombinant RB47 including the NCL did not display endoribonuclease activity in vitro,

245 ease activity in vitro, versions lacking the NCL displayed strong activity.

246 The purpose of making the NCL available is to encourage the use of the NEXUS forma

247 lay phase, indicating a dominant role of the NCL in numerical working memory.

248 The selectivity of the NCL reaction makes in situ membrane formation compatible

249 The findings suggest that the NCL plays a role in learning arbitrary associations, a c

250 tags and protein sequencing showed that the NCL was excised from a full-length recombinant substrate

251 episome binding and maintenance, whereas the NCL C-terminal K380 and K393 induced oriP DNA H3K4me2 mo

252 The NCLs, although relatively rare, share many pathological

253 in vitro and/or in vivo efficacy across the NCLs with an emphasis on targets of action.

254 important early pathological targets in the NCLs and has identified two proteins, VDAC1 and Pttg1, w

255 y onset and rapid disease progression in the NCLs suggests that one or more key cellular processes ar

256 tic axonal and synaptic vulnerability in the NCLs.

257 tic basis and ultrastructural changes in the NCLs.

258 out PPT2 as the causative gene in any of the NCLs at defined chromosomal loci.

259 JNCL is the most common of the NCLs, a group of disorders with infant or childhood onse

260 understand the genetic underpinnings of the NCLs, we performed whole-exome sequencing on DNA samples

261 esis of all 20 natural amino acids and their NCL reactions.

262 Therefore, NCL is an attractive target for antineoplastic treatment

263 Herein, we report that three NCL disease forms with similar tissue pathology are conn

264 in were homogeneously distributed throughout NCL.

265 Binding of 4LB5 to NCL on the cell surface of a variety of breast cancer an

266 any peptide or expressed protein amenable to NCL.

267 re used to identify forebrain projections to NCL and to suggest a possible role of this area in media

268 arcinoma HeLa cells, analysis of a traceable NCL 3'UTR bearing MS2 RNA hairpins revealed that NCL RNA

269 expression profiles existed between the two NCL models studied, 2 of the 15 proteins examined (VDAC1

270 We present a family with typical NCL pathology in which we performed exome sequencing and

271 ed to be orthologous to the genes underlying NCL in nclf mice and in South Hampshire and Merino sheep

272 sights into the mechanisms by which EBV uses NCL and RPL4 to establish persistent B-lymphoblastoid ce

273 gest that selective targeting of KDM1A using NCL-1 and NCD-38 is a promising therapeutic strategy for

274 Pharmacological inhibition of KDM1A using NCL-1 and NCD-38 significantly reduced the cell viabilit

275 knowledge this is the first example of using NCL to generate phospholipids de novo.

276 a region thought to contain the human Type V NCL locus.

277 ic form of Parkinson's disease (PD), whereas NCL is a lysosomal storage disorder.

278 1 binding to oriP and transcription, whereas NCL K429A was deficient.

279 ligomerize, consistent with a model in which NCL stabilizes this double-stranded RNA structure.

280 While NCL is one of the most popular tools for synthesizing pr

281 These findings are consistent with NCL acting as an IRES trans-acting factor (ITAF) for ORF

282 The RPL4 N terminus cooperated with NCL-K429 to support EBNA1 and oriP-mediated episome bind

283 proximately 8% of individuals diagnosed with NCL by conservative clinical and histopathologic criteri

284 al preparation was developed, co-loaded with NCL-240, a small-molecule inhibitor of the PI3K/mTOR pat

285 Neuropathological analysis of patients with NCL shows accumulation of intracellular autofluorescent