Genomes and Genes
Experts and Doctors on small nuclear ribonucleoproteins in Germany
Topic: small nuclear ribonucleoproteins
- Tritschler F, Eulalio A, Truffault V, Hartmann M, Helms S, Schmidt S, et al. A divergent Sm fold in EDC3 proteins mediates DCP1 binding and P-body targeting. Mol Cell Biol. 2007;27:8600-11 pubmed..The conservation of surface and of critical structural residues indicates that LSm domains in EDC3 proteins adopt a similar fold that has separable novel functions that are absent in canonical (L)Sm proteins...
- Wagner F, Poole J, Flegel W. Scianna antigens including Rd are expressed by ERMAP. Blood. 2003;101:752-7 pubmed..Hence, the phenotype prediction by genotyping became possible for all human blood group systems encoded by proteins. ..
- Jablonka S, Holtmann B, Meister G, Bandilla M, Rossoll W, Fischer U, et al. Gene targeting of Gemin2 in mice reveals a correlation between defects in the biogenesis of U snRNPs and motoneuron cell death. Proc Natl Acad Sci U S A. 2002;99:10126-31 pubmed..This finding correlates with enhanced motoneuron degeneration in Gemin2(+/-)/Smn(+/-) mice. Our data provide in vivo evidence that impaired production of U snRNPs contributes to motoneuron degeneration. ..
- Buhler D, Raker V, Luhrmann R, Fischer U. Essential role for the tudor domain of SMN in spliceosomal U snRNP assembly: implications for spinal muscular atrophy. Hum Mol Genet. 1999;8:2351-7 pubmed..Thus, our data show that SMN is an essential U snRNP assembly factor and establish a direct correlation between defects in the biogenesis of U snRNPs and SMA. ..
- Salgado Garrido J, Bragado Nilsson E, Kandels Lewis S, Seraphin B. Sm and Sm-like proteins assemble in two related complexes of deep evolutionary origin. EMBO J. 1999;18:3451-62 pubmed..We also identified archaeal proteins related to Sm and Sm-like proteins. Our results demonstrate that Sm and Sm-like proteins assemble in at least two functionally conserved complexes of deep evolutionary origin. ..
- Muller D, Rehbein M, Baumeister H, Richter D. Molecular characterization of a novel rat protein structurally related to poly(A) binding proteins and the 70K protein of the U1 small nuclear ribonucleoprotein particle (snRNP). Nucleic Acids Res. 1992;20:1471-5 pubmed..Ontogenic studies reveal that the expression of the 100 kDa protein-encoding gene and sexual maturation are correlated, being barely detectable during early post-natal life but reaching maximal levels around the first month after birth. ..
- Riemekasten G, Weiss C, Schneider S, Thiel A, Bruns A, Schumann F, et al. T cell reactivity against the SmD1(83-119) C terminal peptide in patients with systemic lupus erythematosus. Ann Rheum Dis. 2002;61:779-85 pubmed..This strong T cell reactivity as well as the high frequency and specificity of anti-SmD1(83-119) antibodies in SLE suggest a possible role in SLE pathogenesis, at least in a subset of patients. ..
- Gottschalk A, Tang J, Puig O, Salgado J, Neubauer G, Colot H, et al. A comprehensive biochemical and genetic analysis of the yeast U1 snRNP reveals five novel proteins. RNA. 1998;4:374-93 pubmed..Finally, we show that Nam8p/Mud15p contributes to the stability of U1 snRNP. ..
- T r I, Basquin J, Teo Dreher H, Suck D. Archaeal Sm proteins form heptameric and hexameric complexes: crystal structures of the Sm1 and Sm2 proteins from the hyperthermophile Archaeoglobus fulgidus. J Mol Biol. 2002;320:129-42 pubmed publisher..Implications for the functions of archaeal Sm proteins are being discussed...
- Fetzer S, Lauber J, Will C, Luhrmann R. The [U4/U6.U5] tri-snRNP-specific 27K protein is a novel SR protein that can be phosphorylated by the snRNP-associated protein kinase. RNA. 1997;3:344-55 pubmed..Thus, the tri-snRNP-specific 27K protein could potentially be involved in SR protein-mediated protein/protein interactions and, additionally, its phosphorylation state could modulate pre-mRNA splicing. ..
- Watkins N, Gottschalk A, Neubauer G, Kastner B, Fabrizio P, Mann M, et al. Cbf5p, a potential pseudouridine synthase, and Nhp2p, a putative RNA-binding protein, are present together with Gar1p in all H BOX/ACA-motif snoRNPs and constitute a common bipartite structure. RNA. 1998;4:1549-68 pubmed..Electron microscopy of purified snR42 and snR30 particles revealed that these two snoRNPs possess a similar bipartite structure that we propose to be a major structural determining principle for all H/ACA snoRNPs. ..
- Meister G, Eggert C, Buhler D, Brahms H, Kambach C, Fischer U. Methylation of Sm proteins by a complex containing PRMT5 and the putative U snRNP assembly factor pICln. Curr Biol. 2001;11:1990-4 pubmed..Our data suggest that the pICln complex regulates an early step in the assembly of U snRNPs, possibly the transfer of Sm proteins to the SMN-complex. ..
- Förch P, Puig O, Martínez C, Seraphin B, Valcarcel J. The splicing regulator TIA-1 interacts with U1-C to promote U1 snRNP recruitment to 5' splice sites. EMBO J. 2002;21:6882-92 pubmed
- Makarova O, Makarov E, Luhrmann R. The 65 and 110 kDa SR-related proteins of the U4/U6.U5 tri-snRNP are essential for the assembly of mature spliceosomes. EMBO J. 2001;20:2553-63 pubmed..Moreover, since both proteins contain an N-terminal RS domain, they could mediate the association of the tri-snRNP with pre-spliceosomes by interaction with members of the SR protein family. ..
- Sun Y, Grimmler M, Schwarzer V, Schoenen F, Fischer U, Wirth B. Molecular and functional analysis of intragenic SMN1 mutations in patients with spinal muscular atrophy. Hum Mutat. 2005;25:64-71 pubmed..However, all mutations, including those in exon 2a, a region shown to be important for the binding of SMN to SIP1, do not disturb the interaction of SMN to SIP1. ..
- Golas M, Sander B, Will C, Luhrmann R, Stark H. Major conformational change in the complex SF3b upon integration into the spliceosomal U11/U12 di-snRNP as revealed by electron cryomicroscopy. Mol Cell. 2005;17:869-83 pubmed..The manner in which SF3b is integrated in the U11/U12 di-snRNP has important implications for branch site recognition. Furthermore, a putative model of the pre-mRNA binding to the U11/U12 di-snRNP is proposed. ..
- Meister G, Buhler D, Laggerbauer B, Zobawa M, Lottspeich F, Fischer U. Characterization of a nuclear 20S complex containing the survival of motor neurons (SMN) protein and a specific subset of spliceosomal Sm proteins. Hum Mol Genet. 2000;9:1977-86 pubmed..This shows that the SMN-Sm protein interaction is not restricted to the cytoplasm. Our data imply that nuclear SMN affects splicing by modulating the Sm protein composition of U snRNPs. ..
- Winkler C, Eggert C, Gradl D, Meister G, Giegerich M, Wedlich D, et al. Reduced U snRNP assembly causes motor axon degeneration in an animal model for spinal muscular atrophy. Genes Dev. 2005;19:2320-30 pubmed..These findings suggest that motoneuron degeneration in SMA patients is a direct consequence of impaired production of U snRNPs. ..
- Lemm I, Girard C, Kuhn A, Watkins N, Schneider M, Bordonné R, et al. Ongoing U snRNP biogenesis is required for the integrity of Cajal bodies. Mol Biol Cell. 2006;17:3221-31 pubmed..Altogether, our data suggest that CBs have a modular structure with distinct domains for spliceosomal U snRNPs and snoRNPs. ..
- Caspary F, Seraphin B. The yeast U2A'/U2B complex is required for pre-spliceosome formation. EMBO J. 1998;17:6348-58 pubmed..This phenotype can be rescued partially, but specifically, by addition of the corresponding recombinant protein(s). This demonstrates a specific role for the yeast U2 snRNP specific proteins during formation of the pre-spliceosome. ..
- Kuhn A, van Santen M, Schwienhorst A, Urlaub H, Luhrmann R. Stalling of spliceosome assembly at distinct stages by small-molecule inhibitors of protein acetylation and deacetylation. RNA. 2009;15:153-75 pubmed publisher..This suggests that the stalled complexes represent hitherto unobserved intermediates of spliceosome assembly. ..
- Unterholzner L, Izaurralde E. SMG7 acts as a molecular link between mRNA surveillance and mRNA decay. Mol Cell. 2004;16:587-96 pubmed..Our findings indicate that SMG7 provides a link between the NMD and the mRNA degradation machinery by interacting with SMG5 and UPF1 via its N-terminal domain and targeting bound transcripts for decay via its C-terminal domain...
- Buiting K, Dittrich B, Endele S, Horsthemke B. Identification of novel exons 3' to the human SNRPN gene. Genomics. 1997;40:132-7 pubmed
- Sauter C, Basquin J, Suck D. Sm-like proteins in Eubacteria: the crystal structure of the Hfq protein from Escherichia coli. Nucleic Acids Res. 2003;31:4091-8 pubmed..Finally, a detailed structural comparison shows that the Sm-fold is remarkably well conserved in bacteria, Archaea and Eukarya, and represents a universal and modular building unit for oligomeric RNA binding proteins...
- Schultz A, Nottrott S, Watkins N, Luhrmann R. Protein-protein and protein-RNA contacts both contribute to the 15.5K-mediated assembly of the U4/U6 snRNP and the box C/D snoRNPs. Mol Cell Biol. 2006;26:5146-54 pubmed..5K. In conclusion, our data suggest that the formation of each RNP involves the direct recognition of specific elements in both 15.5K protein and the specific RNA. ..
- Dittrich B, Buiting K, Korn B, Rickard S, Buxton J, Saitoh S, et al. Imprint switching on human chromosome 15 may involve alternative transcripts of the SNRPN gene. Nat Genet. 1996;14:163-70 pubmed..This suggests that imprint switching on human chromosome 15 may involve alternative SNRPN transcripts. ..
- Otter S, Grimmler M, Neuenkirchen N, Chari A, Sickmann A, Fischer U. A comprehensive interaction map of the human survival of motor neuron (SMN) complex. J Biol Chem. 2007;282:5825-33 pubmed..Collectively, the data presented here provide a basis for the detailed mechanistic and structural analysis of the assembly machinery of U snRNPs. ..
- Hermann H, Fabrizio P, Raker V, Foulaki K, Hornig H, Brahms H, et al. snRNP Sm proteins share two evolutionarily conserved sequence motifs which are involved in Sm protein-protein interactions. EMBO J. 1995;14:2076-88 pubmed
- Neuenkirchen N, Chari A, Fischer U. Deciphering the assembly pathway of Sm-class U snRNPs. FEBS Lett. 2008;582:1997-2003 pubmed publisher..Here, we summarize recent progress in the understanding of this process and discuss the influence exerted by the aforementioned trans-acting factors. ..
- Biertümpfel C, Basquin J, Suck D, Sauter C. Crystallization of biological macromolecules using agarose gel. Acta Crystallogr D Biol Crystallogr. 2002;58:1657-9 pubmed..This paper gives a brief overview of their general properties and presents an application of a counter-diffusion setup combining agarose gel and capillaries to the crystallization of proteins and protein / nucleic acid complexes...
- Hartmuth K, Urlaub H, Vornlocher H, Will C, Gentzel M, Wilm M, et al. Protein composition of human prespliceosomes isolated by a tobramycin affinity-selection method. Proc Natl Acad Sci U S A. 2002;99:16719-24 pubmed..These data provide insight into the complexity of the splicing machinery at an early stage of its assembly. ..
- Gaubitz M, Wegmann C, Schotte H, Willeke P, Domschke W. Differentiation of RNP- and SM-antibody subsets in SLE and MCTD patients by a new ELISA using recombinant antigens. Cell Mol Biol (Noisy-le-grand). 2002;48:317-21 pubmed..In SLE and MCTD, determination of subsets of antibodies against Ul-snRNP-68 kDa and Sm-complex allows a differentiation of patient subgroups with more definite diagnoses and potential prognostic impact. ..
- Schenkel H, Hanke S, De Lorenzo C, Schmitt R, Mechler B. P elements inserted in the vicinity of or within the Drosophila snRNP SmD3 gene nested in the first intron of the Ornithine Decarboxylase Antizyme gene affect only the expression of SmD3. Genetics. 2002;161:763-72 pubmed..Interestingly, AZ inactivation causes a new phenotype characterized by late larval lethality and atrophy of the brain, imaginal discs, hematopoietic organs, and salivary glands. ..
- 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. ..
- Paraskeva E, Izaurralde E, Bischoff F, Huber J, Kutay U, Hartmann E, et al. CRM1-mediated recycling of snurportin 1 to the cytoplasm. J Cell Biol. 1999;145:255-64 pubmed..This mechanism appears crucial for productive import cycles as it can ensure that CRM1 only exports snurportin 1 that has already released its import substrate in the nucleus. ..
- Selenko P, Sprangers R, Stier G, Buhler D, Fischer U, Sattler M. SMN tudor domain structure and its interaction with the Sm proteins. Nat Struct Biol. 2001;8:27-31 pubmed..Our data provide a structural basis for a molecular defect underlying SMA. ..
- Lehmeier T, Raker V, Hermann H, Luhrmann R. cDNA cloning of the Sm proteins D2 and D3 from human small nuclear ribonucleoproteins: evidence for a direct D1-D2 interaction. Proc Natl Acad Sci U S A. 1994;91:12317-21 pubmed..A., Manolson, M., Becherer, K., Weidenhammer, E., Kirkpatrick, D., Wright, R. & Jones, E. (1991) Mol. Cell. Biol. 11, 5801-5812], suggesting that this gene encodes the yeast homologue of the human D3 protein. ..
- Bouveret E, Rigaut G, Shevchenko A, Wilm M, Seraphin B. A Sm-like protein complex that participates in mRNA degradation. EMBO J. 2000;19:1661-71 pubmed..These results indicate the involvement of a new conserved Sm-like protein complex and a new factor, Pat1p, in mRNA degradation and suggest a physical connection between decapping and exonuclease trimming. ..
- Damianov A, Kann M, Lane W, Bindereif A. Human RBM28 protein is a specific nucleolar component of the spliceosomal snRNPs. Biol Chem. 2006;387:1455-60 pubmed..Our data provide the first evidence that RBM28 is a common nucleolar component of the spliceosomal ribonucleoprotein complexes, possibly coordinating their transition through the nucleolus. ..
- Hetzer M, Mattaj I. An ATP-dependent, Ran-independent mechanism for nuclear import of the U1A and U2B" spliceosome proteins. J Cell Biol. 2000;148:293-303 pubmed..This activity can be solubilized in the presence of elevated MgCl(2). These data suggest that U1A and U2B" import into the nucleus occurs by a hitherto uncharacterized mechanism. ..
- Hirsch E, Oohashi T, Ahmad M, Stamm S, Fassler R. Peri-implantation lethality in mice lacking the Sm motif-containing protein Lsm4. Mol Cell Biol. 2000;20:1055-62 pubmed..The early death of mLsm4p-null mice suggests that the role of mLsm4p in splicing is essential and cannot be compensated by other Lsm proteins. ..
- Nottrott S, Hartmuth K, Fabrizio P, Urlaub H, Vidovic I, Ficner R, et al. Functional interaction of a novel 15.5kD [U4/U6.U5] tri-snRNP protein with the 5' stem-loop of U4 snRNA. EMBO J. 1999;18:6119-33 pubmed..Our finding that the 15.5kD protein also efficiently binds to the 5' stem-loop of U4atac snRNA indicates that it may be shared by the [U4atac/U6atac.U5] tri-snRNP of the minor U12-type spliceosome. ..
- Karaduman R, Fabrizio P, Hartmuth K, Urlaub H, Luhrmann R. RNA structure and RNA-protein interactions in purified yeast U6 snRNPs. J Mol Biol. 2006;356:1248-62 pubmed..Interestingly, we find that the open structure of the yeast U6 snRNA in native snRNPs can also be adopted by human U6 and U6atac snRNAs. ..
- Wilk H, Schaefer K, Agris P, Boak A, Kovacs S. U1 SnRNP association with HnRNP involves an initial non-specific splice-site independent interaction of U1 SnRNP protein with HnRNA. Mol Cell Biochem. 1991;106:55-66 pubmed..This complex may then be further stabilized by intron-specific interactions and hnRNP proteins, as well as by other snRNPs. ..
- Behrens S, Luhrmann R. Immunoaffinity purification of a [U4/U6.U5] tri-snRNP from human cells. Genes Dev. 1991;5:1439-52 pubmed..U5] is phosphorylated only in the latter. The function of this phosphorylation is unclear thus far; it may be involved in the activation of [U4/U6.U5] in the spliceosome. ..
- Puig O, Gottschalk A, Fabrizio P, Seraphin B. Interaction of the U1 snRNP with nonconserved intronic sequences affects 5' splice site selection. Genes Dev. 1999;13:569-80 pubmed..This supports a model where early 5' splice recognition results from a network of interactions established by the splicing machinery with various regions of the pre-mRNA. ..
- Brahms H, Raymackers J, Union A, De Keyser F, Meheus L, Luhrmann R. The C-terminal RG dipeptide repeats of the spliceosomal Sm proteins D1 and D3 contain symmetrical dimethylarginines, which form a major B-cell epitope for anti-Sm autoantibodies. J Biol Chem. 2000;275:17122-9 pubmed
- Urlaub H, Hartmuth K, Kostka S, Grelle G, Luhrmann R. A general approach for identification of RNA-protein cross-linking sites within native human spliceosomal small nuclear ribonucleoproteins (snRNPs). Analysis of RNA-protein contacts in native U1 and U4/U6.U5 snRNPs. J Biol Chem. 2000;275:41458-68 pubmed..In summary, our approach provides a rapid method for identification of RNA-protein contact sites within native snRNP particles as well as other ribonucleoprotein particles. ..
- Vidovic I, Nottrott S, Hartmuth K, Luhrmann R, Ficner R. Crystal structure of the spliceosomal 15.5kD protein bound to a U4 snRNA fragment. Mol Cell. 2000;6:1331-42 pubmed..5kD protein with box C/D snoRNAs. It additionally suggests a general recognition principle in a novel family of RNA binding proteins. ..
- Plessel G, Luhrmann R, Kastner B. Electron microscopy of assembly intermediates of the snRNP core: morphological similarities between the RNA-free (E.F.G) protein heteromer and the intact snRNP core. J Mol Biol. 1997;265:87-94 pubmed
- Schneider C, Will C, BROSIUS J, Frilander M, Luhrmann R. Identification of an evolutionarily divergent U11 small nuclear ribonucleoprotein particle in Drosophila. Proc Natl Acad Sci U S A. 2004;101:9584-9 pubmed..A comparison of U11 snRNAs that we have identified from vertebrates, plants, and insects, suggests that an evolutionarily divergent U11 snRNA may be unique to Drosophila and not characteristic of insects in general. ..
- Urlaub H, Raker V, Kostka S, Luhrmann R. Sm protein-Sm site RNA interactions within the inner ring of the spliceosomal snRNP core structure. EMBO J. 2001;20:187-96 pubmed..Our results thus provide the first evidence that, within the core snRNP, multiple Sm protein-Sm site RNA contacts occur on the inner surface of the heptameric Sm protein ring. ..
- Damianov A, Schreiner S, Bindereif A. Recycling of the U12-type spliceosome requires p110, a component of the U6atac snRNP. Mol Cell Biol. 2004;24:1700-8 pubmed..With a U12-dependent in vitro splicing system, we demonstrate that p110 is required for recycling of the U4atac/U6atac snRNP. ..
- Will C, Schneider C, MacMillan A, Katopodis N, Neubauer G, Wilm M, et al. A novel U2 and U11/U12 snRNP protein that associates with the pre-mRNA branch site. EMBO J. 2001;20:4536-46 pubmed..Immuno precipitations indicated that p14 is present in U12-type spliceosomes, consistent with the idea that branch point selection is similar in the major and minor spliceosomes. ..
- Heinrichs V, Hackl W, Luhrmann R. Direct binding of small nuclear ribonucleoprotein G to the Sm site of small nuclear RNA. Ultraviolet light cross-linking of protein G to the AAU stretch within the Sm site (AAUUUGUGG) of U1 small nuclear ribonucleoprotein reconstituted in vitro. J Mol Biol. 1992;227:15-28 pubmed..These results suggest that the snRNP protein G may be involved in the direct recognition of the Sm site. ..
- Palfi Z, L cke S, Lahm H, Lane W, Kruft V, Bragado Nilsson E, et al. The spliceosomal snRNP core complex of Trypanosoma brucei: cloning and functional analysis reveals seven Sm protein constituents. Proc Natl Acad Sci U S A. 2000;97:8967-72 pubmed publisher..The conservation extends also to the functional level, because at least one trypanosome Sm protein, SmG, was able to specifically complement a corresponding mutation in yeast...
- Grimmler M, Otter S, Peter C, Müller F, Chari A, Fischer U. Unrip, a factor implicated in cap-independent translation, associates with the cytosolic SMN complex and influences its intracellular localization. Hum Mol Genet. 2005;14:3099-111 pubmed..We speculate that unrip plays a crucial role in the intracellular distribution of the SMN complex...
- Strasser A, Dickmanns A, Luhrmann R, Ficner R. Structural basis for m3G-cap-mediated nuclear import of spliceosomal UsnRNPs by snurportin1. EMBO J. 2005;24:2235-43 pubmed..The critical role of this tryptophan and as well of a tryptophan continuing the RNA base stack was confirmed by nuclear import assays and cap-binding activity tests using several snurportin1 mutants. ..
- Czudnochowski N, Vollmuth F, Baumann S, Vogel Bachmayr K, Geyer M. Specificity of Hexim1 and Hexim2 complex formation with cyclin T1/T2, importin alpha and 7SK snRNA. J Mol Biol. 2010;395:28-41 pubmed publisher..These results provide the molecular basis for the generation of a core complex for the inhibition of P-TEFb. ..