Gene Symbol: SNRNP200
Description: small nuclear ribonucleoprotein U5 subunit 200
Alias: ASCC3L1, BRR2, HELIC2, RP33, U5-200KD, U5 small nuclear ribonucleoprotein 200 kDa helicase, BRR2 homolog, U5 snRNP-specific 200 kDa protein, activating signal cointegrator 1 complex subunit 3-like 1, bad response to refrigeration 2 homolog, small nuclear ribonucleoprotein 200kDa (U5), small nuclear ribonucleoprotein, U5 200kDa subunit
Species: human
Products:     SNRNP200

Top Publications

  1. Laggerbauer B, Achsel T, Luhrmann R. The human U5-200kD DEXH-box protein unwinds U4/U6 RNA duplices in vitro. Proc Natl Acad Sci U S A. 1998;95:4188-92 pubmed
    ..The RNA unwinding activity was found to reside exclusively with the U5-200kD DEXH-box protein. Our data raise the interesting possibility that this RNA helicase catalyzes unwinding of the U4/U6 RNA duplex in the spliceosome. ..
  2. Zhao C, Lu S, Zhou X, Zhang X, Zhao K, Larsson C. A novel locus (RP33) for autosomal dominant retinitis pigmentosa mapping to chromosomal region 2cen-q12.1. Hum Genet. 2006;119:617-23 pubmed
    ..Using a genome-wide linkage screening approach, a novel disease locus (RP33) was assigned to the long arm of chromosome 2. A maximum multi-point LOD score of 4...
  3. Pena V, Liu S, Bujnicki J, Luhrmann R, Wahl M. Structure of a multipartite protein-protein interaction domain in splicing factor prp8 and its link to retinitis pigmentosa. Mol Cell. 2007;25:615-24 pubmed
    ..The DEAD-box protein Brr2 and the GTPase Snu114 bind to the Prp8 C terminus, a region where mutations in human Prp8 are linked to the RP13 ..
  4. Liu T, Jin X, Zhang X, Yuan H, Cheng J, Lee J, et al. A novel missense SNRNP200 mutation associated with autosomal dominant retinitis pigmentosa in a Chinese family. PLoS ONE. 2012;7:e45464 pubmed publisher
    The SNRNP200 gene encodes hBrr2, a helicase essential for pre-mRNA splicing. Six mutations in SNRNP200 have recently been discovered to be associated with autosomal dominant retinitis pigmentosa (adRP)...
  5. Li N, Mei H, Macdonald I, Jiao X, Hejtmancik J. Mutations in ASCC3L1 on 2q11.2 are associated with autosomal dominant retinitis pigmentosa in a Chinese family. Invest Ophthalmol Vis Sci. 2010;51:1036-43 pubmed publisher
    ..5 at theta = 0 for D2S2333 and 3.46 at theta = 0 for D2S2216. This region harbors the ASCC3L1 gene. Sequencing of ASCC3L1 in an affected family member showed a heterozygous single-base-pair change; c...
  6. Lauber J, Fabrizio P, Teigelkamp S, Lane W, Hartmann E, Luhrmann R. The HeLa 200 kDa U5 snRNP-specific protein and its homologue in Saccharomyces cerevisiae are members of the DEXH-box protein family of putative RNA helicases. EMBO J. 1996;15:4001-15 pubmed
    ..We propose that U5-200kD and Snu246p promote one or more conformational changes in the dynamic network of RNA-RNA interactions in the spliceosome. ..
  7. Achsel T, Ahrens K, Brahms H, Teigelkamp S, Luhrmann R. The human U5-220kD protein (hPrp8) forms a stable RNA-free complex with several U5-specific proteins, including an RNA unwindase, a homologue of ribosomal elongation factor EF-2, and a novel WD-40 protein. Mol Cell Biol. 1998;18:6756-66 pubmed
    ..Since the 220kD protein is also known to contact both the pre-mRNA and the U5 snRNA, it is in a position to relay the functional state of the spliceosome to the other proteins in the complex and thus modulate their activity. ..
  8. Mozaffari Jovin S, Wandersleben T, Santos K, Will C, Luhrmann R, Wahl M. Inhibition of RNA helicase Brr2 by the C-terminal tail of the spliceosomal protein Prp8. Science. 2013;341:80-4 pubmed publisher
    The Ski2-like RNA helicase Brr2 is a core component of the spliceosome that must be tightly regulated to ensure correct timing of spliceosome activation...
  9. Benaglio P, McGee T, Capelli L, Harper S, Berson E, Rivolta C. Next generation sequencing of pooled samples reveals new SNRNP200 mutations associated with retinitis pigmentosa. Hum Mutat. 2011;32:E2246-58 pubmed publisher
    The gene SNRNP200 is composed of 45 exons and encodes a protein essential for pre-mRNA splicing, the 200 kDa helicase hBrr2...

More Information


  1. Zhao C, Bellur D, Lu S, Zhao F, Grassi M, Bowne S, et al. Autosomal-dominant retinitis pigmentosa caused by a mutation in SNRNP200, a gene required for unwinding of U4/U6 snRNAs. Am J Hum Genet. 2009;85:617-27 pubmed publisher
    ..We recently assigned a locus (RP33) for adRP to 2cen-q12.1, a region that harbors the SNRNP200 gene encoding hBrr2, another U4/U6-U5 snRNP component that is required for unwinding of U4/U6 snRNAs during ..
  2. Liu S, Rauhut R, Vornlocher H, Luhrmann R. The network of protein-protein interactions within the human U4/U6.U5 tri-snRNP. RNA. 2006;12:1418-30 pubmed
    ..Taken together, data presented here provide a detailed picture of the network of protein interactions within the human tri-snRNP. ..
  3. Charenton C, Wilkinson M, Nagai K. Mechanism of 5' splice site transfer for human spliceosome activation. Science. 2019;364:362-367 pubmed publisher
    ..suggest that formation of the 5'SS-ACAGAGA helix triggers remodeling of an intricate protein-RNA network to induce Brr2 helicase relocation to its loading sequence in U4 snRNA, enabling Brr2 to unwind the U4/U6 snRNA duplex to allow U6 ..
  4. Verma S, De Jesus P, Chanda S, Verma I. SNW1, a Novel Transcriptional Regulator of the NF-κB Pathway. Mol Cell Biol. 2019;39: pubmed publisher
    ..We show that SNW1 detaches from its splicing complex (formed with SNRNP200 and SNRNP220) upon NF-κB activation and binds to NF-κB's transcriptional elongation partner p-TEFb...
  5. Ledoux S, Guthrie C. Retinitis Pigmentosa Mutations in Bad Response to Refrigeration 2 (Brr2) Impair ATPase and Helicase Activity. J Biol Chem. 2016;291:11954-65 pubmed publisher
    b>Brr2 is an RNA-dependent ATPase required to unwind the U4/U6 snRNA duplex during spliceosome assembly...
  6. Sticht J, Bertazzon M, Henning L, Licha J, Abualrous E, Freund C. FBP21's C-Terminal Domain Remains Dynamic When Wrapped around the c-Sec63 Unit of Brr2 Helicase. Biophys J. 2019;116:406-418 pubmed publisher
    Based on our recent finding that FBP21 regulates human Brr2 helicase activity involved in the activation of the spliceosomal B-complex, we investigated the structural and dynamic contribution of FBP21 to the interaction...
  7. Zhan X, Yan C, Zhang X, Lei J, Shi Y. Structures of the human pre-catalytic spliceosome and its precursor spliceosome. Cell Res. 2018;28:1129-1140 pubmed publisher
    ..The pre-B-to-B and B-to-Bact transitions are driven by the ATPase/helicases Prp28 and Brr2, respectively...
  8. Weber G, Cristão V, Santos K, Jovin S, Heroven A, Holton N, et al. Structural basis for dual roles of Aar2p in U5 snRNP assembly. Genes Dev. 2013;27:525-40 pubmed publisher assembled via a cytoplasmic precursor that contains the U5-specific Prp8 protein but lacks the U5-specific Brr2 helicase. Instead, pre-U5 snRNP includes the Aar2 protein not found in mature U5 snRNP or spliceosomes...
  9. Wang J, Tadeo X, Hou H, Andrews S, Moresco J, Yates J, et al. Tls1 regulates splicing of shelterin components to control telomeric heterochromatin assembly and telomere length. Nucleic Acids Res. 2014;42:11419-32 pubmed publisher
    ..and mass spectrometry analysis of its interacting proteins show that Tls1 associates with the spliceosome subunit Brr2. RNA sequencing analysis shows that the splicing of a subset of mRNAs are affected in tls1Δ cells, including ..
  10. Agafonov D, Kastner B, Dybkov O, Hofele R, Liu W, Urlaub H, et al. Molecular architecture of the human U4/U6.U5 tri-snRNP. Science. 2016;351:1416-20 pubmed publisher
    ..Our model reveals how the spatial organization of Brr2 RNA helicase prevents premature U4/U6 RNA unwinding in isolated human tri-snRNPs and how the ubiquitin C-terminal ..
  11. Astuti G, van den Born L, Khan M, Hamel C, Bocquet B, Manes G, et al. Identification of Inherited Retinal Disease-Associated Genetic Variants in 11 Candidate Genes. Genes (Basel). 2018;9: pubmed publisher
    ..and dominant inheritance patterns were found for variants in Small Nuclear Ribonucleoprotein U5 Subunit 200 (SNRNP200) and Zinc Finger Protein 513 (ZNF513), respectively...
  12. Xu G, Li T, Chen J, Li C, Zhao H, Yao C, et al. Autosomal dominant retinitis pigmentosa-associated gene PRPF8 is essential for hypoxia-induced mitophagy through regulating ULK1 mRNA splicing. Autophagy. 2018;14:1818-1830 pubmed publisher
    ..Moreover, knockdown of other adRP-associated splicing factors, including PRPF6, PRPF31 and SNRNP200, also lead to ULK1 mRNA mis-splicing and mitophagy defects...
  13. Wollenhaupt J, Henning L, Sticht J, Becke C, Freund C, Santos K, et al. Intrinsically Disordered Protein Ntr2 Modulates the Spliceosomal RNA Helicase Brr2. Biophys J. 2018;114:788-799 pubmed publisher
    ..resonance measurements revealed that Ntr2 uses an N-terminal region to bind the C-terminal helicase unit of the Brr2 RNA helicase, an enzyme involved in spliceosome activation and implicated in splicing catalysis and spliceosome ..
  14. Mayerle M, Guthrie C. Prp8 retinitis pigmentosa mutants cause defects in the transition between the catalytic steps of splicing. RNA. 2016;22:793-809 pubmed publisher
    ..Prp8 and Snu114, an EF2-like GTPase, regulate the activity of the Brr2 helicase, promoting RNA unwinding by Brr2 at appropriate points in the splicing cycle and repressing it at others...
  15. Absmeier E, Wollenhaupt J, Mozaffari Jovin S, Becke C, Lee C, Preussner M, et al. The large N-terminal region of the Brr2 RNA helicase guides productive spliceosome activation. Genes Dev. 2015;29:2576-87 pubmed publisher
    The Brr2 helicase provides the key remodeling activity for spliceosome catalytic activation, during which it disrupts the U4/U6 di-snRNP (small nuclear RNA protein), and its activity has to be tightly regulated...
  16. Liu S, Mozaffari Jovin S, Wollenhaupt J, Santos K, Theuser M, Dunin Horkawicz S, et al. A composite double-/single-stranded RNA-binding region in protein Prp3 supports tri-snRNP stability and splicing. elife. 2015;4:e07320 pubmed publisher
    ..RNA-binding region assembles cooperatively with Snu13 and Prp31 on U4/U6 di-snRNAs and inhibits Brr2-mediated U4/U6 di-snRNA unwinding in vitro...
  17. Liu Y, Chen X, Qin B, Zhao K, Zhao Q, Staley J, et al. Knocking Down Snrnp200 Initiates Demorphogenesis of Rod Photoreceptors in Zebrafish. J Ophthalmol. 2015;2015:816329 pubmed publisher
    Purpose. The small nuclear ribonucleoprotein 200 kDa (SNRNP200) gene is a fundamental component for precursor message RNA (pre-mRNA) splicing and has been implicated in the etiology of autosomal dominant retinitis pigmentosa (adRP)...
  18. Zhang L, Li X, Hill R, Qiu Y, Zhang W, Hansen K, et al. Brr2 plays a role in spliceosomal activation in addition to U4/U6 unwinding. Nucleic Acids Res. 2015;43:3286-97 pubmed publisher
    b>Brr2 is a DExD/H-box RNA helicase that is responsible for U4/U6 unwinding, a critical step in spliceosomal activation...
  19. Absmeier E, Becke C, Wollenhaupt J, Santos K, Wahl M. Interplay of cis- and trans-regulatory mechanisms in the spliceosomal RNA helicase Brr2. Cell Cycle. 2017;16:100-112 pubmed publisher
    RNA helicase Brr2 is implicated in multiple phases of pre-mRNA splicing and thus requires tight regulation...
  20. Rigo N, Sun C, Fabrizio P, Kastner B, Lührmann R. Protein localisation by electron microscopy reveals the architecture of the yeast spliceosomal B complex. EMBO J. 2015;34:3059-73 pubmed publisher
    ..We located the U5 snRNP components Brr2 at the top and Prp8 and Snu114 in the centre of the main body...
  21. Mahrez W, Shin J, Muñoz Viana R, Figueiredo D, Trejo Arellano M, Exner V, et al. BRR2a Affects Flowering Time via FLC Splicing. PLoS Genet. 2016;12:e1005924 pubmed publisher
    ..In a screen for Arabidopsis early flowering mutants we identified an allele of BRR2a. BRR2 proteins are components of the spliceosome and highly conserved in eukaryotes...
  22. Cordin O, Hahn D, Alexander R, Gautam A, Saveanu C, Barrass J, et al. Brr2p carboxy-terminal Sec63 domain modulates Prp16 splicing RNA helicase. Nucleic Acids Res. 2014;42:13897-910 pubmed publisher
    ..Combining mutations in BRR2 and PRP16 suppresses or enhances physical interaction and growth defects in an allele-specific manner, signifying ..
  23. Hahn D, Kudla G, Tollervey D, Beggs J. Brr2p-mediated conformational rearrangements in the spliceosome during activation and substrate repositioning. Genes Dev. 2012;26:2408-21 pubmed publisher
    ..Consistent with this, mutant brr2-G858R reduced second-step splicing efficiency and enhanced cross-linking to 3' ends of introns...
  24. Linder B, Hirmer A, Gal A, Rüther K, Bolz H, Winkler C, et al. Identification of a PRPF4 loss-of-function variant that abrogates U4/U6.U5 tri-snRNP integration and is associated with retinitis pigmentosa. PLoS ONE. 2014;9:e111754 pubmed publisher
    ..Mutations in six spliceosomal proteins, PRPF3, PRPF4, PRPF6, PRPF8, PRPF31 and SNRNP200, cause retinitis pigmentosa (RP), a disease characterized by progressive photoreceptor degeneration...
  25. Cui H, Schlesinger J, Schoenhals S, Tönjes M, Dunkel I, Meierhofer D, et al. Phosphorylation of the chromatin remodeling factor DPF3a induces cardiac hypertrophy through releasing HEY repressors from DNA. Nucleic Acids Res. 2016;44:2538-53 pubmed publisher
    ..Thus, we present a novel pathway for pathological cardiac hypertrophy, whose inhibition is a long-term therapeutic goal for the treatment of the course of heart failure. ..
  26. Theuser M, Hobartner C, Wahl M, Santos K. Substrate-assisted mechanism of RNP disruption by the spliceosomal Brr2 RNA helicase. Proc Natl Acad Sci U S A. 2016;113:7798-803 pubmed publisher
    The Brr2 RNA helicase disrupts the U4/U6 di-small nuclear RNA-protein complex (di-snRNP) during spliceosome activation via ATP-driven translocation on the U4 snRNA strand...
  27. Santos K, Jovin S, Weber G, Pena V, Luhrmann R, Wahl M. Structural basis for functional cooperation between tandem helicase cassettes in Brr2-mediated remodeling of the spliceosome. Proc Natl Acad Sci U S A. 2012;109:17418-23 pubmed publisher
    ..The 250-kDa Brr2 enzyme, which is essential for U4/U6 di-small nuclear ribonucleoprotein disruption during spliceosome catalytic ..
  28. Ito M, Iwatani M, Yamamoto T, Tanaka T, Kawamoto T, Morishita D, et al. Discovery of spiro[indole-3,2'-pyrrolidin]-2(1H)-one based inhibitors targeting Brr2, a core component of the U5 snRNP. Bioorg Med Chem. 2017;25:4753-4767 pubmed publisher
    Bad response to refrigeration 2 (Brr2) is a member of the Ski2-like RNA helicases, and an essential component of the U5 small nuclear ribonucleoprotein (snRNP)...
  29. Niu Z, Jin W, Zhang L, Li X. Tumor suppressor RBM5 directly interacts with the DExD/H-box protein DHX15 and stimulates its helicase activity. FEBS Lett. 2012;586:977-83 pubmed publisher
    ..Helicase activities play critical roles in modulating pre-mRNA splicing. Our findings thus suggest a new mechanism underlying the regulatory roles of RBM5 in pre-mRNA splicing. ..
  30. Ehsani A, Alluin J, Rossi J. Cell cycle abnormalities associated with differential perturbations of the human U5 snRNP associated U5-200kD RNA helicase. PLoS ONE. 2013;8:e62125 pubmed publisher
    ..One of the key components of the tri-snRNP is the U5 200kd helicase...
  31. Malinová A, Cvačková Z, Matějů D, Hořejší Z, Abéza C, Vandermoere F, et al. Assembly of the U5 snRNP component PRPF8 is controlled by the HSP90/R2TP chaperones. J Cell Biol. 2017;216:1579-1596 pubmed publisher
    ..The pre-mRNA splicing factor PRPF8 is a crucial component of the U5 snRNP, and together with EFTUD2 and SNRNP200, it forms a central module of the spliceosome...
  32. Bowne S, Sullivan L, Avery C, Sasser E, Roorda A, Duncan J, et al. Mutations in the small nuclear riboprotein 200 kDa gene (SNRNP200) cause 1.6% of autosomal dominant retinitis pigmentosa. Mol Vis. 2013;19:2407-17 pubmed
    The purpose of this project was to determine the spectrum and frequency of mutations in the small nuclear riboprotein 200 kDa gene (SNRNP200) that cause autosomal dominant retinitis pigmentosa (adRP).
  33. Cvacková Z, Matějů D, Stanek D. Retinitis pigmentosa mutations of SNRNP200 enhance cryptic splice-site recognition. Hum Mutat. 2014;35:308-17 pubmed publisher
    ..The protein product of SNRNP200 is BRR2, a DExD/H box RNA helicase crucial for pre-mRNA splicing. In this study, we prepared p.S1087L and p...
  34. Velasco K, Zhao B, Callegari S, Altun M, Liu H, Hassink G, et al. An N-terminal SIAH-interacting motif regulates the stability of the ubiquitin specific protease (USP)-19. Biochem Biophys Res Commun. 2013;433:390-5 pubmed publisher
    ..The interaction is mediated by a SIAH-consensus binding motif and promotes USP19 ubiquitylation and proteasome-dependent degradation. These findings identify USP19 as a common substrate of the SIAH ubiquitin ligases. ..
  35. Escher P, Passarin O, Munier F, Tran V, Vaclavik V. Variability in clinical phenotypes of PRPF8-linked autosomal dominant retinitis pigmentosa correlates with differential PRPF8/SNRNP200 interactions. Ophthalmic Genet. 2018;39:80-86 pubmed publisher
    ..All reported disease-causing PRPF8 variants were collected and localized in the PRPF8 and PRPF8/SNRNP200 protein structures. The p...
  36. Peng R, Dye B, Pérez I, Barnard D, Thompson A, Patton J. PSF and p54nrb bind a conserved stem in U5 snRNA. RNA. 2002;8:1334-47 pubmed
    ..Sedimentation analyses show that both proteins associate with spliceosomes and with U4/U6.U5 tri-snPNP. ..
  37. Vertegaal A, Ogg S, Jaffray E, Rodriguez M, Hay R, Andersen J, et al. A proteomic study of SUMO-2 target proteins. J Biol Chem. 2004;279:33791-8 pubmed
    ..SART1 and heterogeneous nuclear RNP M were both shown to be genuine SUMO targets, confirming the validity of the approach. ..
  38. Guo R, Zheng L, Park J, Lv R, Chen H, Jiao F, et al. BS69/ZMYND11 reads and connects histone H3.3 lysine 36 trimethylation-decorated chromatin to regulated pre-mRNA processing. Mol Cell. 2014;56:298-310 pubmed publisher
    ..3K36me3-specific reader and a regulator of IR and reveals that BS69 connects histone H3.3K36me3 to regulated RNA splicing, providing significant, important insights into chromatin regulation of pre-mRNA processing. ..
  39. Plaschka C, Lin P, Nagai K. Structure of a pre-catalytic spliceosome. Nature. 2017;546:617-621 pubmed publisher
    ..between U4/U6 di-snRNP and the U2 snRNP SF3b-containing domain, which also transiently contacts the helicase Brr2. The 3' region of the U2 snRNP is flexibly attached to the SF3b-containing domain and protrudes over the concave ..
  40. Henning L, Santos K, Sticht J, Jehle S, Lee C, Wittwer M, et al. A new role for FBP21 as regulator of Brr2 helicase activity. Nucleic Acids Res. 2017;45:7922-7937 pubmed publisher
    ..which we identified several spliceosomal binding partners in a yeast-two-hybrid screen, among them the RNA helicase Brr2. Biochemical and biophysical analyses revealed that an intrinsically disordered region of FBP21 binds to an ..
  41. Huang L, Xiao X, Li S, Jia X, Wang P, Sun W, et al. Molecular genetics of cone-rod dystrophy in Chinese patients: New data from 61 probands and mutation overview of 163 probands. Exp Eye Res. 2016;146:252-8 pubmed publisher
    ..2%), UNC119 (0.6%), RPGRIP1 (1.2%), RDH12 (0.6%), KCNV2 (0.6%), C21orf2 (0.6%), CEP290 (0.6%), USH2A (0.6%) and SNRNP200 (0.6%)...
  42. Zhang X, Lai T, Chiang S, Tam P, Liu D, Chan C, et al. Contribution of SNRNP200 sequence variations to retinitis pigmentosa. Eye (Lond). 2013;27:1204-13 pubmed publisher
    Mutations in the SNRNP200 gene have been reported to cause autosomal dominant retinitis pigmentosa (adRP). In this study, we evaluate the mutation profile of SNRNP200 in a cohort of southern Chinese RP patients.
  43. Pabis M, Neufeld N, Steiner M, Bojic T, Shav Tal Y, Neugebauer K. The nuclear cap-binding complex interacts with the U4/U6·U5 tri-snRNP and promotes spliceosome assembly in mammalian cells. RNA. 2013;19:1054-63 pubmed publisher
    ..Taken together, the data support a model whereby CBC promotes pre-mRNA splicing through a network of interactions with and among spliceosomal snRNPs during cotranscriptional spliceosome assembly. ..
  44. Tremblay N, Baril M, Chatel Chaix L, Es Saad S, Park A, Koenekoop R, et al. Spliceosome SNRNP200 Promotes Viral RNA Sensing and IRF3 Activation of Antiviral Response. PLoS Pathog. 2016;12:e1005772 pubmed publisher
    Spliceosomal SNRNP200 is a Ski2-like RNA helicase that is associated with retinitis pigmentosa 33 (RP33)...
  45. Meister G, Hannus S, Plöttner O, Baars T, Hartmann E, Fakan S, et al. SMNrp is an essential pre-mRNA splicing factor required for the formation of the mature spliceosome. EMBO J. 2001;20:2304-14 pubmed
    ..We suggest that SMNrp, as a U2 snRNP-associated protein, facilitates the recruitment of the [U4/U6.U5] tri-snRNP to the pre-spliceosome. ..
  46. Sato N, Maeda M, Sugiyama M, Ito S, Hyodo T, Masuda A, et al. Inhibition of SNW1 association with spliceosomal proteins promotes apoptosis in breast cancer cells. Cancer Med. 2015;4:268-77 pubmed publisher
    ..analyses revealed that SNW1 directly associates with other spliceosome components, including EFTUD2 (Snu114) and SNRNP200 (Brr2)...
  47. Kotlajich M, Hertel K. Death by splicing: tumor suppressor RBM5 freezes splice-site pairing. Mol Cell. 2008;32:162-4 pubmed publisher
    ..In a recent issue of Molecular Cell, Bonnal et al. (2008) demonstrate that the tumor suppressor gene RBM5 regulates alternative splicing of Fas pre-mRNA by interfering with splice-site pairing. ..
  48. Schneider C, Will C, Makarova O, Makarov E, Luhrmann R. Human U4/U6.U5 and U4atac/U6atac.U5 tri-snRNPs exhibit similar protein compositions. Mol Cell Biol. 2002;22:3219-29 pubmed
  49. Laggerbauer B, Lauber J, Luhrmann R. Identification of an RNA-dependent ATPase activity in mammalian U5 snRNPs. Nucleic Acids Res. 1996;24:868-75 pubmed
    ..Furthermore, both the formation of the crosslink and ATPase activity appear with a similar substrate specificity for ATP. ..
  50. Bertram K, Agafonov D, Dybkov O, Haselbach D, Leelaram M, Will C, et al. Cryo-EM Structure of a Pre-catalytic Human Spliceosome Primed for Activation. Cell. 2017;170:701-713.e11 pubmed publisher
    ..that creates, together with Dim1, a 5' splice site (ss) binding pocket, displacement of Sad1, and rearrangement of Brr2 such that it contacts its U4/U6 substrate and is poised for the subsequent spliceosome activation step...
  51. Gillissen M, Kedde M, de Jong G, Moiset G, Yasuda E, Levie S, et al. AML-specific cytotoxic antibodies in patients with durable graft versus leukemia responses. Blood. 2017;: pubmed publisher
    ..A number of these donor-derived antibodies recognized the U5 snRNP200 complex, a component of the spliceosome that in normal cells is found in the cell...