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1 snRNA quality control may also be relevant in spinal mus
2 NA (different subunits) and 5250 miRNA, 3747 snRNA, gene sequences from 9282 complete genome chromoso
3 n (snRNP), which contains, additionally, 7SK snRNA, methyl phosphate-capping enzyme (MePCE), and La-r
4 ID alone in cells dissociates HEXIM1 and 7SK snRNA from P-TEFb, but it is not sufficient to activate
6 H4R3me(2(s)), which is directly read by 7SK snRNA, and decapping/demethylation of 7SK snRNA, ensurin
8 SK snRNA, and decapping/demethylation of 7SK snRNA, ensuring the dismissal of the 7SK snRNA/HEXIM inh
16 east, the U2 small nuclear ribonucleic acid (snRNA) component of the spliceosome is targeted for addi
17 riptional modification of U56 and U93 alters snRNA conformational dynamics by distinct mechanisms and
20 oding RNAs such as tRNAs, snoRNAs, rRNAs and snRNAs preferentially produce small 5' and 3' end fragme
21 ence for small RNA genes (tRNAs, snoRNAs and snRNAs) suggesting a putative role for RNA in its recrui
24 sites of most mRNA genes but are present at snRNA genes and the highly transcribed heat shock genes
27 tion of almost any integrator subunit causes snRNA misprocessing, very little is known about the role
32 o increases the levels of assembly-defective snRNAs and suppresses some splicing defects seen in SMN-
34 antling of the Prp3-binding site on U4/U6 di-snRNA but leaves the Prp31- and Snu13-binding sites on U
37 sing factor 31 (Prp31), and Prp3 to U4/U6 di-snRNA leads to a stepwise decrease of Brr2-mediated U4/U
39 yses that Prp3 contains a bipartite U4/U6 di-snRNA-binding region comprising an expanded ferredoxin-l
42 operatively with Snu13 and Prp31 on U4/U6 di-snRNAs and inhibits Brr2-mediated U4/U6 di-snRNA unwindi
43 strongly inhibited by mutations in U4/U6 di-snRNAs that diminish the ability of U6 snRNA to adopt an
45 n of these interaction domains alone elicits snRNA misprocessing through a dominant-negative titratio
46 s ptRNA-subclasses that exist in eukaryotes: snRNA, snoRNA, RNase P, RNase MRP, Y RNA or telomerase R
51 be both necessary and nearly sufficient for snRNA biogenesis in cells depleted of endogenous IntS12
52 de evidence for the production of functional snRNAs by Wolbachia that play roles in cross-kingdom com
54 ction as transcription terminators for human snRNA genes with little, if any, role in snRNA 3'-end pr
55 1 and U2 genes as models, we show that human snRNA genes are more similar to mRNA genes than yeast sn
57 Production of snR-DPGs required the Pol II snRNA promoter (PIIsnR), and CPL4RNAi plants showed incr
66 A turnover at short transcription units like snRNA-, replication-dependent histone-, promoter upstrea
68 monstrated that a new class of human U1-like snRNAs, the variant (v)U1 snRNAs (vU1s), also participat
71 we first demonstrate that yeast U1 Sm-mutant snRNAs are degraded either by Rrp6- or by Dcp2-dependent
73 operate to protect and chaperone the nascent snRNA during its journey to the spliceosome.The mechanis
76 for introns and various RNA classes (ncRNA, snRNA, snoRNA) and less variability after degradation.
77 single cells (scRNA-seq) and single nuclei (snRNA-seq) and found them comparable, with a distinct en
79 rocessing of snRNAs, increases the levels of snRNA primary transcripts (pre-snRNAs), and alters the o
80 se results identify a conserved mechanism of snRNA quality control, and also suggest a general paradi
81 that LEC functions in at least two phases of snRNA transcription: an initiation step requiring the IC
84 ecreases the occupancy of LEC at a subset of snRNA genes and results in a reduction in their transcri
85 ole in the recruitment of LEC to a subset of snRNA genes through direct interaction of EAF and the N-
87 a hypomorphic phenotype, as only a subset of snRNA transcripts are quantitatively altered in snapc4(s
89 esults indicate that during transcription of snRNA genes, Ser7 phosphorylation facilitates recruitmen
93 ding to snRNAs are known to reduce levels of snRNAs, suggesting an unknown quality control system for
96 al defects, impairs the 3' end processing of snRNAs, increases the levels of snRNA primary transcript
97 pts correlated with coilin ChIP-seq peaks on snRNA genes, indicating that coilin binding to nascent s
99 ssembly factor for loading the Sm complex on snRNAs and, when severely reduced, can lead to reduced l
100 faithful delivery of seven Sm proteins onto snRNA and the formation of the common core of snRNPs.
102 complex with the Sm site or m(7)G cap of pre-snRNA, which reveal that the WD40 domain of Gemin5 recog
103 vels of TOE1 accumulated 3'-end-extended pre-snRNAs, and the immunoisolated TOE1 complex was sufficie
104 nd sufficient for binding the Sm site of pre-snRNAs by isothermal titration calorimetry (ITC) and mut
105 recognizes the Sm site and m(7)G cap of pre-snRNAs via two distinct binding sites by respective base
108 complex delivers pre-small nuclear RNAs (pre-snRNAs) to the heptameric Sm ring for the assembly of th
110 the levels of snRNA primary transcripts (pre-snRNAs), and alters the occupancy of Pol II at snRNA loc
119 ture of low-abundance U12 small nuclear RNA (snRNA) in Arabidopsis thaliana and provide in vivo evide
122 Mg(2+) site in the U2/U6 small nuclear RNA (snRNA) triplex, and the 5'-phosphate of the intron nucle
123 etween coilin and rRNA, U small nuclear RNA (snRNA), and human telomerase RNA, which is altered upon
124 3' end of spliceosomal U6 small nuclear RNA (snRNA), directly catalyzing terminal 2', 3' cyclic phosp
125 mal complex comprising U5 small nuclear RNA (snRNA), extensively base-paired U4/U6 snRNAs and more th
126 RNP), composed of the 7SK small nuclear RNA (snRNA), MePCE, and Larp7, regulates the mRNA elongation
130 on a functional U2 snRNP (small nuclear RNA [snRNA] plus associated proteins), as H2A.Z shows extensi
132 xpression of modified U1 small nuclear RNAs (snRNA) complementary to the splice donor sites strongly
135 tion of 3'-extensions of small nuclear RNAs (snRNAs) and biogenesis of novel transcripts from protein
136 sembles from five U-rich small nuclear RNAs (snRNAs) and over 200 proteins in a highly dynamic fashio
138 NAPII for transcription, small nuclear RNAs (snRNAs) display a further requirement for a factor known
140 ntified in the U4 and U6 small nuclear RNAs (snRNAs) suggest U4/U6 stem I as a Brr2p substrate during
141 rsors to specific tRNAs, small nuclear RNAs (snRNAs), and small nucleolar RNAs (snoRNAs) are all enri
142 usly proposed the use of small nuclear RNAs (snRNAs), especially U7snRNA to shuttle the antisense seq
143 compartments enriched in small nuclear RNAs (snRNAs)-and promotes efficient spliceosomal snRNP assemb
147 whole-cell and single-nuclei RNA-sequencing (snRNA-seq) methods, here we show that snRNA-seq faithful
153 ncing (snRNA-seq) methods, here we show that snRNA-seq faithfully recapitulates transcriptional patte
154 udy identifies a complex responsible for the snRNA 3' end maturation in plants and uncovers a previou
156 In the wild type, salt stress induced the snRNA-to-snR-DPG switch, which was associated with alter
157 plants showed increased read-through of the snRNA 3'-end processing signal, leading to continuation
159 ell Integrator complex, which recognizes the snRNA 3' end processing signal (3' box), generates the 5
160 ive binding of all protein components to the snRNA duplex during di-snRNP assembly by electrophoretic
162 from protein-coding genes downstream of the snRNAs (snRNA-downstream protein-coding genes [snR-DPGs]
164 ar splicing speckles and associates with the snRNAs that are involved in splice site recognition.
166 se of a neurodegenerative syndrome linked to snRNA maturation and uncover a key factor involved in th
167 Defects in Sm protein complex binding to snRNAs are known to reduce levels of snRNAs, suggesting
169 plants, suggesting that the transcriptional snRNA-to-snR-DPG switch may be a ubiquitous mechanism to
170 erns similar to canonical ncRNAs (e.g. tRNA, snRNA, miRNA, etc) on approximately 70% of human long nc
173 ites and modest delays at some histone and U snRNA genes, suggesting that the torpedo mechanism is no
177 to the decline in the cytosolic levels of U snRNAs and of the SMN complex proteins SMN and DDX20 tha
178 ial infection resulted in the rerouting of U snRNAs and their cytoplasmic escort, the survival motor
180 than an overall reduction in Uridyl-rich (U)-snRNAs, may contribute to the specific neuromuscular dis
181 Although uridine-rich small nuclear RNAs (U-snRNAs) are essential for pre-mRNA splicing, little is k
182 Strikingly, we have been unable to find a U1 snRNA candidate or any predicted U1-associated proteins,
187 In addition, disruption of the Prp8 and U1 snRNA interaction reduces tri-snRNP level in the spliceo
188 e U1 snRNP core particle (Sm proteins and U1 snRNA), but not the mature U1 snRNP-specific proteins (U
189 contacts between the 5' splice site-bound U1 snRNA and neighboring exonic sequences that, in turn, in
191 find that URB binds human and Drosophila U1 snRNA SLII and U2 snRNA SLIV with higher affinity than d
192 gether, these data suggest that the human U1 snRNA variants analyzed here are unable to efficiently b
193 s the 3' introns, compensatory changes in U1 snRNA rescue trans-splicing of TSA mutants, demonstratin
196 omplex (CBC) to the 5' end of the nascent U1 snRNA, which ultimately influences the utilization of U1
198 rpholino that base-pairs to the 5' end of U1 snRNA blocks splicing in the coupled system and complete
204 ticles (snRNPs) that are comprised of the U1 snRNA and 10 core components, including U1A, U1-70K, U1C
205 e found that this interaction between the U1 snRNA and SF3A1 occurs within prespliceosomal complexes
207 the AU dincucleotide at the 5'-end of the U1 snRNA is highly conserved, despite the absence of an app
209 eraction between stem-loop 4 (SL4) of the U1 snRNA, which recognizes the 5' splice site, and a compon
210 , we present a mutational analysis of the U1 snRNA, which shows that although enlarging the 5' leader
214 ed predominantly through basepairing with U1 snRNA whilst U1-C fine-tunes relative affinities of mism
218 We show that Cr1-activating engineered U1 snRNAs (eU1s) have the unique ability to reprogram pre-m
220 one case, we also evaluated exon-specific U1 snRNAs that, by targeting nonconserved intronic sequence
221 s of human U1-like snRNAs, the variant (v)U1 snRNAs (vU1s), also participate in pre-mRNA processing e
224 terminal RNA recognition motif of p65, a U12 snRNA binding protein, also binds to the distal 3' stem-
225 s of proteins on 25S rRNA, 5.8S rRNA and U12 snRNA structure, illustrating the critical importance of
226 ian U12 snRNAs, the loop of the SLIIb in U12 snRNA is variable among plant species, and DMS/SHAPE-LMP
227 p65 protein-binding apical stem-loop of U12 snRNA can be replaced by this U6atac distal 3' stem-loop
228 ecular helix I region between U6atac and U12 snRNAs, several other regions within these RNA molecules
229 Interestingly, in contrast to mammalian U12 snRNAs, the loop of the SLIIb in U12 snRNA is variable a
230 ds human and Drosophila U1 snRNA SLII and U2 snRNA SLIV with higher affinity than do modern homologs,
232 the context of TER1 suggests that the BS-U2 snRNA interaction is disrupted after the first step and
233 icates that Prp5 has reduced affinity for U2 snRNA that lacks Psi42 and Psi44 and that Prp5 ATPase ac
234 er, our results indicate that the Psis in U2 snRNA contribute to pre-mRNA splicing by directly alteri
239 Nase activity within the CU region of the U2 snRNA primary transcript in vitro, and that coilin knock
244 k-turns from ribosomes, riboswitches and U4 snRNA, finding a strong conservation of properties for a
245 d predispose them to ion-induced folding, U4 snRNA are strongly biased to an inability to such foldin
249 ffold during the entire assembly, but the U4 snRNA 5' stem-loop adopts alternative orientations each
250 anslocates only a limited distance on the U4 snRNA strand and does not actively release RNA-bound pro
253 he amino-terminal domain of Prp8 position U5 snRNA to insert its loop I, which aligns the exons for s
254 mutations in PRP16, PRP8, SNU114 and the U5 snRNA that affect this process interact genetically with
256 ruitment, packaging of both pre-tRNAs and U6 snRNA requires the nuclear export receptor Exportin-5.
258 e observations suggest that the conserved U6 snRNA methyltransferase evolved an additional function i
259 of the same gRNA expressed from different U6 snRNA promoters, with the previously untested U6:3 promo
260 then completed by the partially displaced U6 snRNA adopting an alternative conformation, which leads
263 es downstream.We show that the 3' ends of U6 snRNA in PN patient lymphoblasts are elongated and unexp
264 ta implicate aberrant oligoadenylation of U6 snRNA in the pathogenesis of the leukemia predisposition
265 U6 di-snRNAs that diminish the ability of U6 snRNA to adopt an alternative conformation but leave the
266 e fold, which recognizes a 3'-overhang of U6 snRNA, and a preceding peptide, which binds U4/U6 stem I
268 tional 3'-end processing by USB1 protects U6 snRNA from targeting and destruction by the nuclear exos
269 the tri-snRNP and comparison with a Prp24-U6 snRNA recycling complex suggests how Prp3 may be involve
270 can activate the spliceosome by stripping U6 snRNA of all precatalytic binding partners, while minimi
274 from budding yeast, here we show that the U6 snRNA catalyses both of the two splicing reactions by po
276 yeasts rely on hyperstabilization of the U6 snRNA-5' splice site interaction to impede the 2nd step
280 e ability of Delta247-Brr2 to bind the U4/U6 snRNA duplex at high pH and increases Delta247-Brr2's RN
282 snRNA between its 3' stem-loop and the U4/U6 snRNA stem I is loaded into the Brr2 helicase active sit
283 ss known direct Prp8-binding sites on U5, U6 snRNA and intron-containing pre-mRNAs identified using s
284 n Prp16 stabilizes Cwc2 interactions with U6 snRNA and destabilizes Cwc2 interactions with pre-mRNA,
285 Prp8 directly cross-links with U2, U5 and U6 snRNAs and pre-mRNA in purified activated spliceosomes,
289 P contains extensively base paired U4 and U6 snRNAs, Snu13, Prp31, Prp3 and Prp4, seven Sm and seven
290 r RNA (snRNA), extensively base-paired U4/U6 snRNAs and more than 30 proteins, including the key comp
291 n, leading to a dramatic reduction of U5, U6 snRNAs and accumulation of U1 snRNA in the B(act) comple
293 rated that the 3' stem-loop region of U6atac snRNA contains a U12-dependent spliceosome-specific targ
294 pressed, it is unclear whether these variant snRNAs have the capacity to form snRNPs and participate
295 ique ribonucleoproteins (RNPs), and many vU1 snRNA genes are differentially expressed in human embryo
296 ides to block the activity of a specific vU1 snRNA in HeLa cells, we have identified global transcrip
299 omal activation through its interaction with snRNAs and possibly other spliceosomal proteins, reveali
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