IndraLab

Statements

PRPF8 affects SNRNP200
17 | 21 26
PRPF8 binds SNRNP200.
17 | 11 23
SNRNP200 binds PRPF8. 10 / 38
17 | 9 12
sparser
"Furthermore, TSSC4 directly interacts with the SNRNP200 (U5 snRNP 200 kDa helicase) and the PRPF8 spliceosomal scaffold."
39697342
sparser
"For example, pathogenic variants in PRPF8 and SNRNP200 were clustered in their interaction regions, where defects disrupted PRPF8–SNRNP200 interactions. xref – xref Previously, situations similar to that of TOPORS have been reported in the RP1 gene, in which causative heterozygous truncating variants are only located in a specific region in the middle of the gene. xref – xref Regarding TOPORS , products resulting from pathogenic truncating variants located in the last exon and downstream of the SR/RS domain may escape nonsense-mediated mRNA decay (NMD) xref and reduce the activity of the wild-type allele via the dominant-negative effect."
35579903
reach
"In the nucleus, PRPF8 is forming a complex with EFTUD2 and SNRNP200 (also known as BRR2) as part of the spliceosome."
34951455
sparser
"In addition, a small but reproducible reduction of SNRNP200 and PRPF8 binding was also observed in the case of the Δ201–250 deletion mutant."
34131137
reach
"These Prp8-RP mutants directly affect U5 snRNP and tri-snRNP maturation (Boon et al. 2007), Brr2 helicase, and ATPase activities (Pena et al. 2007; Maeder et al. 2009; Mozaffari-Jovin et al. 2013), and yeast-two-hybrid interactions between Brr2, Prp8, and Snu114 (Table 1; Pena et al. 2007)."
26968627
reach
"The conservative missense variant p.PRPF8-Arg2310Lys that is not altering the global charge of the C-terminal tail, and variants located at the basis of the C-terminal tail show milder clinical phenotypes, in accordance with functional data on PRPF8 and SNRNP200 interactions in yeast."
29087248
sparser
"It suggests that the C-terminal tail of PRPF8, which is inserted into and blocks the RNA tunnel in SNRNP200, does not considerably contribute to the interaction between PRPF8 and SNRNP200 and overall stability of the U5 snRNP ( xref )."
37019475
sparser
"BRR2 interacts extensively with the U5-specific protein PRPF8 [ xref ]."
28076437
sparser
"SNRNP200 tightly interacts with the splicing factor PRPF8, and these two proteins have been shown to assemble in the cytoplasm and be transported to the nucleus ( xref )."
37333282
sparser
"All the known variants cluster in the C-terminal Jab1/MPN domain of the PRPF8 protein which interacts with SNRNP200 ( xref ; xref ; xref ; xref ; xref ; xref )."
33584830
PRPF8 binds EFTUD2 and SNRNP200. 7 / 7
| 1 6
sparser
"Remarkably, the GFP-ZNHIT2 SILAC IP revealed a strong association of this factor with EFTUD2, PRPF8, and SNRNP200."
28515276
sparser
"Specifically, TSSC4 interacts with U5-specific proteins PRPF8, EFTUD2 and SNRNP200."
34131137
sparser
"Importantly, our results suggest that components of Complex B, and more specifically BRR2 and EFTUD2, which interact directly with PRPF8 to form the U5 snRNP complex [ xref ], phenocopy PRPF8 most strongly."
26392272
sparser
"AAR2, ZNHIT2, and the HSP90/R2TP complex associate with the U5-specific proteins EFTUD2, PRPF8, and SNRNP200."
28515276
sparser
"Similarly, in humans, yeast two-hybrid and in vitro binding assays have demonstrated physical interactions between EFTUD2, SNRNP200 and PRPF8 proteins ( xref , xref ), and these interactions have been confirmed by recent cryo-EM structures of the human U4/U6."
33584830
sparser
"Notably, depletion of the spliceosome components BRR2 and EFTUD2, which interact directly with PRPF8 to form the U5 snRNP [ xref ], most strongly phenocopy PRPF8 deficiency (Fig.  xref ; Additional file xref b, c)."
26392272
reach
"In addition to the PRPF8, EFTUD2, and SNRNP200 complex, our proteomics analysis as well as previous analyses found that SNW1 was in complex with another splicing subcomplex, PRP19 complex."
25450007
sparser
"Because Snrnp40 is tightly bound to Prpf8, Eftud2, and Snrnp200, it is likely that Snrnp40 also participates and facilitates these processes."
31427773
SNRNP200 binds PRPF8 and COPS5. 1 / 1
| 1
sparser
"A recent 7Å cryo-electron microscopy (cryo-EM) structure of the human tri-snRNP revealed that SNRNP200 interacts with the PRPF8 Jab1 domain, the region of PRPF8 containing the majority of PRPF8 RP-linked variants ( xref ; xref )."
33584830
sparser
"Hypomorphic mutations in the core spliceosomal components PRPF3, PRPF8, PRPF31, and SNRNP200 are associated with autosomal dominant retinitis pigmentosa, a disease characterized by progressive retinal degeneration."
22819011
PRPF8 binds SNRNP200 and TSSC4. 1 / 1
| 1
sparser
"The tethering assay (Fig.  xref ) shows that TSSC4 binding to PRPF8, SNRNP200, and EFTUP2 can occur already in the cytoplasm, which suggests that TSSC4 interaction with RHC components happens primarily during their biogenesis."
34131137
EFTUD2 binds SNRNP200, LAP, and PRPF8. 1 / 1
| 1
sparser
"The proteomic analysis of the GFP IPs showed a strong association of PRPF8-LAP with its partners EFTUD2 and SNRNP200, and conversely, GFP-EFTUD2 strongly bound PRPF8 and SNRNP200."
28515276
PRPF8 binds SNRNP200 and PRP8 domains. 1 / 1
| 1
reach
"TSSC4 thereby prevents stable docking of BRR2 on the U5 snRNP but may assist with anchoring the BRR2 to the U5 snRNP, acting jointly with the PRP8-bound BRR2 N-terminus and the PRP8 domains (Fig. 1b-f, Extended Data Fig. 3)."
38467876
PRPF8 inhibits SNRNP200.
| 5 3
PRPF8 inhibits SNRNP200. 7 / 7
| 4 3
reach
"A C-terminal fragment of Prp8 was shown to inhibit Brr2 ATPase activity in vitro [ 35 ]."
22464735
sparser
"Given the mechanistic analogy between histone mRNA processing and splicing, we thus envisage that SLBP ubiquitylation may suppress hPrp43 helicase activity in histone mRNA processing similar to Prp8 u[MISSING/INVALID CREDENTIALS: limited to 200 char for Elsevier]"
27203182
reach
"These proteins show multiple strong genetic (Kuhn et al. 2002; Bottner et al. 2005; Grainger and Beggs 2005; Liu et al. 2006), yeast-two-hybrid (van Nues and Beggs 2001), and physical interactions (Achsel et al. 1998), indicating a high degree of coordination between them.Initial genetic evidence indicated that the Prp8 C-terminal Jab1/MPN domain negatively regulated Brr2 unwinding during splicing (Kuhn and Brow 2000; Kuhn et al. 2002)."
26968627
reach
"Thus Prp8-RP mutations provide a potent tool to understand how deregulation of Brr2 by Prp8 impacts splicing.Here we present evidence that Prp8-RP mutants deregulate Brr2 activity both in vitro and in vivo."
26968627
sparser
"A model was proposed in which inhibition of Brr2 by the ubiquitinated Prp8 leads to spliceosome assembly and stabilization of the triple snRNP (U4/U6–U5)."
| PMC
sparser
"In humans, phosphorylation of Prp6 that occurs after the tri-snRNP being integrated into the B-complex may release the inhibition of Brr2 by Prp8 and is important for spliceosomal B-complex activation [ xref , xref ]."
27058959
reach
"The RNase H domain of PRPF8 inhibits loading of SNRNP200 to U4 snRNA, and a C-terminal part of PRPF8 modulates the SNRNP200 activity for the unwinding of U4/U6 snRNA duplex XREF_BIBR - XREF_BIBR."
25450007
Mutated PRPF8 inhibits mutated SNRNP200. 1 / 1
| 1
reach
"In particular, prp8 mutants suppress brr2 mutants ( Kuhn et al., 2002 ), leading to the hypothesis that Prp8p controls Brr2p (see also Achsel et al. [1998] ), although these data do not rule out a fun[MISSING/INVALID CREDENTIALS: limited to 200 char for Elsevier]"
16885028
PRPF8 activates SNRNP200.
| 5
PRPF8 activates SNRNP200. 5 / 5
| 5
reach
"The carboxy‐terminal region of the spliceosomal protein PRPF8, which modulates the RNA helicase Brr2, is a mutation hotspot linked to retinitis pigmentosa‐type 13, although its precise function in human splicing and tissue‐specific mechanisms remains unclear."
40066647
reach
"Although it has been demonstrated that the PRPF8 RNase H domain inhibits SNRNP200 activity and GTP bound EFTUD2 and that the C-terminus of PRPF8 induces the activation of SNRNP200 XREF_BIBR - XREF_BIBR, the detailed molecular mechanisms of the activation of SNRNP200 remains to be determined."
25450007
reach
"Prp8, which directly regulates Brr2 activity (Maeder et al. 2009; Nielsen and Staley 2012; Mozaffari-Jovin et al. 2013; Nguyen et al. 2013), has also been implicated in 3′SS selection (Umen and Guthrie 1995a,b, 1996; Siatecka et al. 1999) as well as the transition between the first and second catalytic steps (Frank et al. 1992; Query and Konarska 2004; Liu et al. 2007)."
26968627
reach
"Indeed a C-terminal fragment of Prp8 — encompassing the RNaseH-like and Jab/MPN1-like domains — strongly stimulates Brr2-dependent U4/U6 unwinding in vitro [ 44 ]."
20089394
reach
"The RNase H domain of PRPF8 inhibits loading of SNRNP200 to U4 snRNA, and a C-terminal part of PRPF8 modulates the SNRNP200 activity for the unwinding of U4/U6 snRNA duplex XREF_BIBR - XREF_BIBR."
25450007
SNRNP200 affects PRPF8
17 | 12 23
SNRNP200 binds PRPF8.
17 | 11 23
SNRNP200 binds PRPF8. 10 / 38
17 | 9 12
sparser
"Furthermore, TSSC4 directly interacts with the SNRNP200 (U5 snRNP 200 kDa helicase) and the PRPF8 spliceosomal scaffold."
39697342
sparser
"For example, pathogenic variants in PRPF8 and SNRNP200 were clustered in their interaction regions, where defects disrupted PRPF8–SNRNP200 interactions. xref – xref Previously, situations similar to that of TOPORS have been reported in the RP1 gene, in which causative heterozygous truncating variants are only located in a specific region in the middle of the gene. xref – xref Regarding TOPORS , products resulting from pathogenic truncating variants located in the last exon and downstream of the SR/RS domain may escape nonsense-mediated mRNA decay (NMD) xref and reduce the activity of the wild-type allele via the dominant-negative effect."
35579903
reach
"In the nucleus, PRPF8 is forming a complex with EFTUD2 and SNRNP200 (also known as BRR2) as part of the spliceosome."
34951455
sparser
"In addition, a small but reproducible reduction of SNRNP200 and PRPF8 binding was also observed in the case of the Δ201–250 deletion mutant."
34131137
reach
"These Prp8-RP mutants directly affect U5 snRNP and tri-snRNP maturation (Boon et al. 2007), Brr2 helicase, and ATPase activities (Pena et al. 2007; Maeder et al. 2009; Mozaffari-Jovin et al. 2013), and yeast-two-hybrid interactions between Brr2, Prp8, and Snu114 (Table 1; Pena et al. 2007)."
26968627
reach
"The conservative missense variant p.PRPF8-Arg2310Lys that is not altering the global charge of the C-terminal tail, and variants located at the basis of the C-terminal tail show milder clinical phenotypes, in accordance with functional data on PRPF8 and SNRNP200 interactions in yeast."
29087248
sparser
"It suggests that the C-terminal tail of PRPF8, which is inserted into and blocks the RNA tunnel in SNRNP200, does not considerably contribute to the interaction between PRPF8 and SNRNP200 and overall stability of the U5 snRNP ( xref )."
37019475
sparser
"BRR2 interacts extensively with the U5-specific protein PRPF8 [ xref ]."
28076437
sparser
"SNRNP200 tightly interacts with the splicing factor PRPF8, and these two proteins have been shown to assemble in the cytoplasm and be transported to the nucleus ( xref )."
37333282
sparser
"All the known variants cluster in the C-terminal Jab1/MPN domain of the PRPF8 protein which interacts with SNRNP200 ( xref ; xref ; xref ; xref ; xref ; xref )."
33584830
PRPF8 binds EFTUD2 and SNRNP200. 7 / 7
| 1 6
sparser
"Remarkably, the GFP-ZNHIT2 SILAC IP revealed a strong association of this factor with EFTUD2, PRPF8, and SNRNP200."
28515276
sparser
"Specifically, TSSC4 interacts with U5-specific proteins PRPF8, EFTUD2 and SNRNP200."
34131137
sparser
"Importantly, our results suggest that components of Complex B, and more specifically BRR2 and EFTUD2, which interact directly with PRPF8 to form the U5 snRNP complex [ xref ], phenocopy PRPF8 most strongly."
26392272
sparser
"AAR2, ZNHIT2, and the HSP90/R2TP complex associate with the U5-specific proteins EFTUD2, PRPF8, and SNRNP200."
28515276
sparser
"Similarly, in humans, yeast two-hybrid and in vitro binding assays have demonstrated physical interactions between EFTUD2, SNRNP200 and PRPF8 proteins ( xref , xref ), and these interactions have been confirmed by recent cryo-EM structures of the human U4/U6."
33584830
sparser
"Notably, depletion of the spliceosome components BRR2 and EFTUD2, which interact directly with PRPF8 to form the U5 snRNP [ xref ], most strongly phenocopy PRPF8 deficiency (Fig.  xref ; Additional file xref b, c)."
26392272
reach
"In addition to the PRPF8, EFTUD2, and SNRNP200 complex, our proteomics analysis as well as previous analyses found that SNW1 was in complex with another splicing subcomplex, PRP19 complex."
25450007
sparser
"Because Snrnp40 is tightly bound to Prpf8, Eftud2, and Snrnp200, it is likely that Snrnp40 also participates and facilitates these processes."
31427773
SNRNP200 binds PRPF8 and COPS5. 1 / 1
| 1
sparser
"A recent 7Å cryo-electron microscopy (cryo-EM) structure of the human tri-snRNP revealed that SNRNP200 interacts with the PRPF8 Jab1 domain, the region of PRPF8 containing the majority of PRPF8 RP-linked variants ( xref ; xref )."
33584830
sparser
"Hypomorphic mutations in the core spliceosomal components PRPF3, PRPF8, PRPF31, and SNRNP200 are associated with autosomal dominant retinitis pigmentosa, a disease characterized by progressive retinal degeneration."
22819011
PRPF8 binds SNRNP200 and TSSC4. 1 / 1
| 1
sparser
"The tethering assay (Fig.  xref ) shows that TSSC4 binding to PRPF8, SNRNP200, and EFTUP2 can occur already in the cytoplasm, which suggests that TSSC4 interaction with RHC components happens primarily during their biogenesis."
34131137
EFTUD2 binds SNRNP200, LAP, and PRPF8. 1 / 1
| 1
sparser
"The proteomic analysis of the GFP IPs showed a strong association of PRPF8-LAP with its partners EFTUD2 and SNRNP200, and conversely, GFP-EFTUD2 strongly bound PRPF8 and SNRNP200."
28515276
PRPF8 binds SNRNP200 and PRP8 domains. 1 / 1
| 1
reach
"TSSC4 thereby prevents stable docking of BRR2 on the U5 snRNP but may assist with anchoring the BRR2 to the U5 snRNP, acting jointly with the PRP8-bound BRR2 N-terminus and the PRP8 domains (Fig. 1b-f, Extended Data Fig. 3)."
38467876
SNRNP200 deubiquitinates PRPF8.
| 1
SNRNP200 deubiquitinates PRPF8. 1 / 1
| 1
reach
"Following de-ubiquitination of Prp8, Brr2 is free to promote unwinding of U4/U6, resulting in activation of the spliceosome [48]."
| PMC
PLIN2 affects PRPF3, PRPF31, PRPF8, REG3A, SNRNP200, and TOPORS
| 1
PRPF8 binds PRPF31, PRPF3, REG3A, TOPORS, SNRNP200, and PLIN2. 1 / 1
| 1
sparser
"Among the genes involved in mRNA splicing, mutations in PRPC8 (human homolog of yeast pre-mRNA splicing factor C8), PRP31 (human homolog of yeast pre-mRNA splicing factor 31), HPRP3 (human homolog of yeast pre-mRNA splicing factor 3), PAP-1 (PIM-1 kinase), TOPORS (topoisomerase-I-binding arginine/serine-rich protein) and SNRNP200 (small nuclear ribonucleoprotein, 200 kDa) are associated with adRP [ xref - xref ], although the mechanisms behind the process remain unclear."
20519033
PLIN2 affects PRPF3, PRPF31, PRPF8, REG3A, and SNRNP200
| 1
sparser
"Other essential components of the spliceosome, PRPF31, PRPF3, PRPF8, PAP1 and SNRNP200 , have been associated with adRP [ xref - xref ]."
21496248
LAP affects SNRNP200
| 1
EFTUD2 binds SNRNP200, LAP, and PRPF8. 1 / 1
| 1
sparser
"The proteomic analysis of the GFP IPs showed a strong association of PRPF8-LAP with its partners EFTUD2 and SNRNP200, and conversely, GFP-EFTUD2 strongly bound PRPF8 and SNRNP200."
28515276
EFTUD2 affects SNRNP40
| 1
sparser
"Because Snrnp40 is tightly bound to Prpf8, Eftud2, and Snrnp200, it is likely that Snrnp40 also participates and facilitates these processes."
31427773
EFTUD2 affects LAP, PRPF8, and SNRNP200
| 1
EFTUD2 binds SNRNP200, LAP, and PRPF8. 1 / 1
| 1
sparser
"The proteomic analysis of the GFP IPs showed a strong association of PRPF8-LAP with its partners EFTUD2 and SNRNP200, and conversely, GFP-EFTUD2 strongly bound PRPF8 and SNRNP200."
28515276
COPS5 affects SNRNP200
| 1
SNRNP200 binds PRPF8 and COPS5. 1 / 1
| 1
sparser
"A recent 7Å cryo-electron microscopy (cryo-EM) structure of the human tri-snRNP revealed that SNRNP200 interacts with the PRPF8 Jab1 domain, the region of PRPF8 containing the majority of PRPF8 RP-linked variants ( xref ; xref )."
33584830
sparser
"Hypomorphic mutations in the core spliceosomal components PRPF3, PRPF8, PRPF31, and SNRNP200 are associated with autosomal dominant retinitis pigmentosa, a disease characterized by progressive retinal degeneration."
22819011
AAR2 affects ECD, EFTUD2, PRP8, PRPF8, and SNRNP200
| 1
ECD binds PRPF8, PRP8, AAR2, SNRNP200, and EFTUD2. 1 / 1
| 1
sparser
"A recent study by Claudius et al demonstrated that human ECD can rescue splicing defects in drosophila induced by deletion of fly Ecd and Ecd also interacts with a complex containing Prp8, Aar2, Brr2 and Snu114 proteins [ xref ]."
28492064