Gene Symbol: SNRNP70
Description: small nuclear ribonucleoprotein U1 subunit 70
Alias: RNPU1Z, RPU1, SNRP70, Snp1, U1-70K, U170K, U1AP, U1RNP, U1 small nuclear ribonucleoprotein 70 kDa, U1 snRNP 70 kDa, small nuclear ribonucleoprotein 70kDa (U1), small nuclear ribonucleoprotein, U1 70kDa subunit
Species: human
Products:     SNRNP70

Top Publications

  1. Klein Gunnewiek J, van Aarssen Y, van der Kemp A, Nelissen R, Pruijn G, van Venrooij W. Nuclear accumulation of the U1 snRNP-specific protein C is due to diffusion and retention in the nucleus. Exp Cell Res. 1997;235:265-73 pubmed
    ..laevis oocytes and cultured HeLa cells mutant U1C proteins that are not able to bind to the U1 snRNP do not accumulate in the nucleus, indicating that nuclear accumulation of U1C is due to incorporation of the protein into the U1 snRNP. ..
  2. Cho S, Hoang A, Sinha R, Zhong X, Fu X, Krainer A, et al. Interaction between the RNA binding domains of Ser-Arg splicing factor 1 and U1-70K snRNP protein determines early spliceosome assembly. Proc Natl Acad Sci U S A. 2011;108:8233-8 pubmed publisher
  3. Query C, Bentley R, Keene J. A common RNA recognition motif identified within a defined U1 RNA binding domain of the 70K U1 snRNP protein. Cell. 1989;57:89-101 pubmed
  4. Spritz R, Strunk K, Surowy C, Hoch S, Barton D, Francke U. The human U1-70K snRNP protein: cDNA cloning, chromosomal localization, expression, alternative splicing and RNA-binding. Nucleic Acids Res. 1987;15:10373-91 pubmed
    ..This suggests that multiple forms of the hU1-70K protein may exist, possibly with different functions in vivo. ..
  5. Cao W, Garcia Blanco M. A serine/arginine-rich domain in the human U1 70k protein is necessary and sufficient for ASF/SF2 binding. J Biol Chem. 1998;273:20629-35 pubmed
    ..It has been clearly documented that the arginine/serine domain of ASF/SF2 is responsible for binding to the U1 70k protein...
  6. Greidinger E, Foecking M, Ranatunga S, Hoffman R. Apoptotic U1-70 kd is antigenically distinct from the intact form of the U1-70-kd molecule. Arthritis Rheum. 2002;46:1264-9 pubmed
    ..Immunity to apoptotically modified proteins can develop against caspase-cleaved forms or against forms that undergo additional posttranslational modification. ..
  7. Dieker J, Cisterna B, Monneaux F, Decossas M, van der Vlag J, Biggiogera M, et al. Apoptosis-linked changes in the phosphorylation status and subcellular localization of the spliceosomal autoantigen U1-70K. Cell Death Differ. 2008;15:793-804 pubmed publisher
  8. Wu J, Maniatis T. Specific interactions between proteins implicated in splice site selection and regulated alternative splicing. Cell. 1993;75:1061-70 pubmed
  9. Wang H, Lin W, Dyck J, Yeakley J, Songyang Z, Cantley L, et al. SRPK2: a differentially expressed SR protein-specific kinase involved in mediating the interaction and localization of pre-mRNA splicing factors in mammalian cells. J Cell Biol. 1998;140:737-50 pubmed SR protein (ASF/SF2) by either SRPK1 or 2 enhanced its interaction with another RS domain-containing protein (U1 70K), and overexpression of either kinase induced specific redistribution of splicing factors in the nucleus...

More Information


  1. Bai B, Hales C, Chen P, GOZAL Y, Dammer E, Fritz J, et al. U1 small nuclear ribonucleoprotein complex and RNA splicing alterations in Alzheimer's disease. Proc Natl Acad Sci U S A. 2013;110:16562-7 pubmed publisher
    ..Thus, our results demonstrate unique U1 snRNP pathology and implicate abnormal RNA splicing in AD pathogenesis. ..
  2. Betteridge Z, Tansley S, Shaddick G, Chinoy H, Cooper R, New R, et al. Frequency, mutual exclusivity and clinical associations of myositis autoantibodies in a combined European cohort of idiopathic inflammatory myopathy patients. J Autoimmun. 2019;: pubmed publisher
    ..15). Interstitial lung disease was strongly associated with the anti-tRNA synthetases, anti-MDA5, and anti-U1RNP/Sm. Overlap disease was strongly associated with anti-PMScl, anti-Ku, anti-U1RNP/Sm and anti-Ro60...
  3. Zhao C, Zhu L, Li R, Wang H, Cai Z. Omics approach reveals metabolic disorders associated with the cytotoxicity of airborne particulate matter in human lung carcinoma cells. Environ Pollut. 2019;246:45-52 pubmed publisher
    ..up-regulated serine/arginine-rich splicing factor 1 (SRSF1), SRSF2, small nuclear ribonucleoprotein 70 kDa (snRNP70), small nuclear ribonucleoprotein polypeptide B (SNRPB), SNRPC, SNRPE and down-regulated heterogeneous nuclear ..
  4. Kajiwara Y, Franciosi S, Takahashi N, Krug L, Schmeidler J, Taddei K, et al. Extensive proteomic screening identifies the obesity-related NYGGF4 protein as a novel LRP1-interactor, showing reduced expression in early Alzheimer's disease. Mol Neurodegener. 2010;5:1 pubmed publisher
    ..This is particularly interesting in light of the dual role of ApoE in both cardiovascular risk and AD. The results support further studies on the functional relationship between NYGGF4 and LRP1. ..
  5. Martínez Barrio J, Ovalles Bonilla J, López Longo F, González C, Montoro M, Valor L, et al. Juvenile, adult and late-onset systemic lupus erythematosus: a long term follow-up study from a geographic and ethnically homogeneous population. Clin Exp Rheumatol. 2015;33:788-94 pubmed
    ..Low serum complement, anti-dsDNA, anti-U1RNP and anti-Sm antibodies were more common in the juvenile-onset group...
  6. Calise S, Zheng B, Hasegawa T, Satoh M, Isailovic N, Ceribelli A, et al. Reference standards for the detection of anti-mitochondrial and anti-rods/rings autoantibodies. Clin Chem Lab Med. 2018;: pubmed publisher
    ..also produced a nuclear homogenous staining pattern by HEp-2-IFA, immunoprecipitated proteins associated with anti-U1RNP antibody and reacted weakly with histones, nucleosomes, Sm and nRNP/Sm by LIA...
  7. Priora M, Manetta T, Scarati M, Parisi S, Laganà A, Peroni C, et al. Serological and clinical profile of systemic sclerosis: analysis in a cohort of patients from a single center in Northern Italy. G Ital Dermatol Venereol. 2018;153:33-38 pubmed publisher
    ..presence of AntiScl70 correlated with cardiac involvement (arrhythmias, pericarditis and myocarditis) and the Anti-U1RNP correlated with the presence of skin ulcers...
  8. Zhang W, Zhang C, Chen P, Yang C, Gan X, Hussain M, et al. Circulation autoantibody against Lamin A/C in patients with Sjögren's syndrome. Oncotarget. 2016;7:80252-80261 pubmed publisher
    ..As a result, we found that Lamin A/C has similar epitopes with U1RNP. It means that the potential relationship exist between Lamin A/C and U1RNP...
  9. Nunes J, Cunha A, Meirinhos T, Nunes A, Araújo P, Godinho A, et al. Prevalence of auto-antibodies associated to pulmonary arterial hypertension in scleroderma - A review. Autoimmun Rev. 2018;17:1186-1201 pubmed publisher
    ..anti-bicaudal D2, anti-nucleolar, anti-Scl-70 (anti-topoisomerase I), anti-topoisomerase II α, anti-RNP, anti-U1RNP, anti-U3RNP, anti-RNA polymerase III, anti-Th/To, anti-histone, antiphospholipid, anti-PmScl, anti-Sm, anti SSA (..
  10. Bishof I, Dammer E, Duong D, Kundinger S, Gearing M, Lah J, et al. RNA-binding proteins with basic-acidic dipeptide (BAD) domains self-assemble and aggregate in Alzheimer's disease. J Biol Chem. 2018;293:11047-11066 pubmed publisher
    The U1 small nuclear ribonucleoprotein 70 kDa (U1-70K) and other RNA-binding proteins (RBPs) are mislocalized to cytoplasmic neurofibrillary Tau aggregates in Alzheimer's disease (AD), yet the co-aggregation mechanisms are incompletely ..
  11. Gao J, Diao H, Liu Y, Lv M, Dong H, Zhang X, et al. Analysis of immunity index and immunopathogenesis pattern of lupus nephritis patients. J Biol Regul Homeost Agents. 2015;29:201-6 pubmed
    Joint detection of anti-dsDNA antibodies, anti-U1RNP, anti-SM antibodies, anti-SSA antibodies, anti-ribosomal P protein antibodies, anti-nucleosome antivodies (Anua), anti-histone antibodies (AHA) and antinuclear antibodies brings to the ..
  12. Li S, Liu S, Chen F, Cheng Y, Bai R, Lai A, et al. Link-Polymorphism of 5-HTT Promoter Region Is Associated with Autoantibodies in Patients with Systemic Lupus Erythematosus. J Immunol Res. 2016;2016:3042726 pubmed
    ..suggest that the frequencies of SS genotype and S allele in SLE patients with positive anti-Sm antibody and anti-U1RNP antibody were both significantly higher than the other genotypes and alleles...
  13. Parente Costa L, Bonfa E, Martinago C, de Oliveira R, Carvalho J, Pereira R. Juvenile onset Systemic Lupus Erythematosus thyroid dysfunction: a subgroup with mild disease?. J Autoimmun. 2009;33:121-4 pubmed publisher
    ..6% TPOAb and 3.9% TRAb. JOSLE patients with positive thyroid autoantibodies had higher frequency of anti-U1RNP antibodies than patients without these antibodies (40.9 vs. 14.5%, OR:0.25, CI:0.08-0.76, p = 0.017)...
  14. Blencowe B, Issner R, Nickerson J, Sharp P. A coactivator of pre-mRNA splicing. Genes Dev. 1998;12:996-1009 pubmed
    ..The results suggest that a complex of the nuclear matrix proteins SRm160 and SRm300 functions as a coactivator of pre-mRNA splicing. ..
  15. Adams D, van der Weyden L, Mayeda A, Stamm S, Morris B, Rasko J. ZNF265--a novel spliceosomal protein able to induce alternative splicing. J Cell Biol. 2001;154:25-32 pubmed
    ..Alignment with other RS domain-containing proteins revealed a high degree of SR dipeptide conservation. These data show that ZNF265 functions as a novel component of the mRNA processing machinery. ..
  16. Ibarra M, Rigsby C, Morgan G, Sammet C, Huang C, Xu D, et al. Monitoring change in volume of calcifications in juvenile idiopathic inflammatory myopathy: a pilot study using low dose computed tomography. Pediatr Rheumatol Online J. 2016;14:64 pubmed
    ..Ten JIIM, mean age 14.54?±?4.54 years, had a duration of untreated disease of 8.68?±?5.65 months  MSA status: U1RNP (1), PM-Scl (1), Ro (1, 4 indeterminate), p155/140 (2), MJ (3), Mi-2 indeterminate (1), negative (3)...
  17. Hernandez H, Makarova O, Makarov E, Morgner N, Muto Y, Krummel D, et al. Isoforms of U1-70k control subunit dynamics in the human spliceosomal U1 snRNP. PLoS ONE. 2009;4:e7202 pubmed publisher
    ..These results therefore provide the important functional link between proteomics and structure as well as insight into the dynamic quaternary structure of the native U1 snRNP important for its function...
  18. Zhang W, Wu J. Functional properties of p54, a novel SR protein active in constitutive and alternative splicing. Mol Cell Biol. 1996;16:5400-8 pubmed
    ..These findings and the differential tissue distribution of p54 suggest that this novel SR protein may participate in regulation of alternative splicing in a tissue- and substrate-dependent manner. ..
  19. Hube F, Ulveling D, Sureau A, Forveille S, Francastel C. Short intron-derived ncRNAs. Nucleic Acids Res. 2017;45:4768-4781 pubmed publisher
    ..tested their dependence on each key factor in canonical or alternative miRNAs biogenesis (Drosha, DGCR8, DBR1, snRNP70, U2AF65, PRP8, Dicer, Ago2)...
  20. Hales C, Seyfried N, Dammer E, Duong D, Yi H, Gearing M, et al. U1 small nuclear ribonucleoproteins (snRNPs) aggregate in Alzheimer's disease due to autosomal dominant genetic mutations and trisomy 21. Mol Neurodegener. 2014;9:15 pubmed publisher
    ..Since dominant genetic mutations and trisomy 21 result in dysfunctional amyloid processing, the findings suggest that aberrant ?-amyloid processing may influence U1 snRNP aggregate formation. ..
  21. Charroux B, Pellizzoni L, Perkinson R, Yong J, Shevchenko A, Mann M, et al. Gemin4. A novel component of the SMN complex that is found in both gems and nucleoli. J Cell Biol. 2000;148:1177-86 pubmed
    ..Interestingly, Gemin4 is also detected in the nucleoli, suggesting that the SMN complex may also function in preribosomal RNA processing or ribosome assembly. ..
  22. Hinchcliff M, Khanna S, Hsu V, Lee J, Almagor O, Chang R, et al. Survival in systemic sclerosis-pulmonary arterial hypertension by serum autoantibody status in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry. Semin Arthritis Rheum. 2015;45:309-14 pubmed publisher
    ..or non-specific autoantibodies/other, and a combined group with similar survival consisting of RNA polymerase III, U1RNP, and autoantibody-negative patients)...
  23. Rahman M, Azuma Y, Nasrin F, Takeda J, Nazim M, Bin Ahsan K, et al. SRSF1 and hnRNP H antagonistically regulate splicing of COLQ exon 16 in a congenital myasthenic syndrome. Sci Rep. 2015;5:13208 pubmed publisher
    ..Isolation of early spliceosome complex revealed that the mutation impairs binding of U1-70K (snRNP70) to the downstream 5' splice site...
  24. Zhao J, Wang Q, Liu Y, Tian Z, Guo X, Wang H, et al. Clinical characteristics and survival of pulmonary arterial hypertension associated with three major connective tissue diseases: A cohort study in China. Int J Cardiol. 2017;236:432-437 pubmed publisher
    ..4years, and patients with SLE were youngest at the time of PAH diagnosis. The most prevalent autoantibody was anti-U1RNP antibody (55.8%)...
  25. Luo W, Ji Z, Pan Z, You B, Hoque M, Li W, et al. The conserved intronic cleavage and polyadenylation site of CstF-77 gene imparts control of 3' end processing activity through feedback autoregulation and by U1 snRNP. PLoS Genet. 2013;9:e1003613 pubmed publisher
    ..Thus, the conserved intronic pA of the CstF-77 gene may function as a sensor for cellular C/P and splicing activities, controlling the homeostasis of CstF-77 and C/P activity and impacting cell proliferation and differentiation. ..
  26. Monneaux F, Lozano J, Patarroyo M, Briand J, Muller S. T cell recognition and therapeutic effect of a phosphorylated synthetic peptide of the 70K snRNP protein administered in MR/lpr mice. Eur J Immunol. 2003;33:287-96 pubmed
    ..We further demonstrated that P140 is recognized by antibodies from lupus patients and binds to various HLA DR molecules, offering new hope for manipulating T cell response in humans...
  27. Izmirly P, Halushka M, Rosenberg A, Whelton S, Rais Bahrami K, Nath D, et al. Clinical and pathologic implications of extending the spectrum of maternal autoantibodies reactive with ribonucleoproteins associated with cutaneous and now cardiac neonatal lupus from SSA/Ro and SSB/La to U1RNP. Autoimmun Rev. 2017;16:980-983 pubmed publisher
    ..Moreover, the cutaneous manifestations of neonatal lupus have also been identified in infants exposed only to anti-U1RNP antibodies...
  28. Lee C. RH70, a bidirectional RNA helicase, co-purifies with U1snRNP. J Biol Chem. 2002;277:39679-83 pubmed
    ..Its association with U1snRNP and ATP specificity suggest a role for RH70 in pre-mRNA splicing, in particular, at the early stages of the splicing reaction involving U1snRNP. ..
  29. Diner I, Nguyen T, Seyfried N. Enrichment of Detergent-insoluble Protein Aggregates from Human Postmortem Brain. J Vis Exp. 2017;: pubmed publisher
    ..Proteomic analysis also revealed enrichment of β-amyloid (Aβ), tau, snRNP70 (U1-70K), and apolipoprotein E (APOE) in the sarkosyl-insoluble fractions of AD brain compared to those of ..
  30. Tubery A, Fortenfant F, Combe B, Abreu I, Bossuyt X, Chretien P, et al. Clinical association of mixed connective tissue disease and granulomatosis with polyangiitis: a case report and systematic screening of anti-U1RNP and anti-PR3 auto-antibody double positivity in ten European hospitals. Immunol Res. 2016;64:1243-1246 pubmed
    ..of a 50-years-old man treated for mixed connective tissue disease (MCTD) positive for anti-U1 ribonucleoprotein (U1RNP) antibodies who secondarily developed a granulomatosis with polyangiitis (GPA) associated with anti-proteinase 3 ..
  31. Ajuh P, Chusainow J, Ryder U, Lamond A. A novel function for human factor C1 (HCF-1), a host protein required for herpes simplex virus infection, in pre-mRNA splicing. EMBO J. 2002;21:6590-602 pubmed
    ..The inhibition of splicing in tsBN67 cells correlates with the temperature-sensitive cell cycle arrest phenotype, suggesting that HCF-1-dependent splicing events may be required for cell cycle progression. ..
  32. Greidinger E, Zang Y, Jaimes K, Martinez L, Nassiri M, Hoffman R. CD4+ T cells target epitopes residing within the RNA-binding domain of the U1-70-kDa small nuclear ribonucleoprotein autoantigen and have restricted TCR diversity in an HLA-DR4-transgenic murine model of mixed connective tissue disease. J Immunol. 2008;180:8444-54 pubmed
  33. Tian H. RNA ligands generated against complex nuclear targets indicate a role for U1 snRNP in co-ordinating transcription and RNA splicing. FEBS Lett. 2001;509:282-6 pubmed
    ..In addition, U1 small nuclear ribonucleoprotein particles (snRNP) and pol II had been co-immunoprecipitated in the absence of transcription. These results suggest a role for U1 snRNP in co-ordinating transcription and RNA splicing. ..
  34. Tani C, Carli L, Vagnani S, Talarico R, Baldini C, Mosca M, et al. The diagnosis and classification of mixed connective tissue disease. J Autoimmun. 2014;48-49:46-9 pubmed publisher
    ..advances have been made also in disease pathogenesis understanding and a central pathogenetic role of anti-U1RNP autoantibodies has clearly emerged...
  35. Hong W, Bennett M, Xiao Y, Feld Kramer R, Wang C, Reed R. Association of U2 snRNP with the spliceosomal complex E. Nucleic Acids Res. 1997;25:354-61 pubmed
    ..However, we find that U2 snRNP is not as tightly bound in the E complex as it is in the A complex. The possible significance of the weak association of U2 snRNP with the E complex is discussed. ..
  36. Carlson S, Moore K, Sankaran S, Reynoird N, Elias J, Gozani O. A Proteomic Strategy Identifies Lysine Methylation of Splicing Factor snRNP70 by the SETMAR Enzyme. J Biol Chem. 2015;290:12040-7 pubmed publisher
    ..Our approach identified lysine 130 of the mRNA splicing factor snRNP70 as a SETMAR substrate in vitro, and we show that the enzyme primarily generates monomethylation at this position...
  37. Zhang W, Wu J. Sip1, a novel RS domain-containing protein essential for pre-mRNA splicing. Mol Cell Biol. 1998;18:676-84 pubmed
    ..Together, these results demonstrate that Sip1 is a novel RS domain-containing protein required for pre-mRNA splicing and that the functional role of Sip1 in splicing is distinct from those of known RS domain-containing splicing factors. ..
  38. Du Clos T. C-reactive protein reacts with the U1 small nuclear ribonucleoprotein. J Immunol. 1989;143:2553-9 pubmed
    ..Considering the ability of CRP to inhibit antibody responses to its ligands and its ability to activate C and promote phagocytosis it is suggested that CRP may play a role in the regulation of autoantibody responses to nuclear Ag. ..
  39. Akker S, Misra S, Aslam S, Morgan E, Smith P, Khoo B, et al. Pre-spliceosomal binding of U1 small nuclear ribonucleoprotein (RNP) and heterogenous nuclear RNP E1 is associated with suppression of a growth hormone receptor pseudoexon. Mol Endocrinol. 2007;21:2529-40 pubmed
    ..Immunoprecipitation experiments with three other pseudoexons suggested that pre-spliceosomal binding of U1 snRNP is a potential general mechanism of suppression of pseudoexons. ..
  40. Pellizzoni L, Baccon J, Rappsilber J, Mann M, Dreyfuss G. Purification of native survival of motor neurons complexes and identification of Gemin6 as a novel component. J Biol Chem. 2002;277:7540-5 pubmed
    ..Co-immunoprecipitation, immunolocalization, and in vitro binding experiments demonstrate that Gemin6 is a component of the SMN complex that localizes to gems and interacts with several Sm proteins of the spliceosomal snRNPs. ..
  41. Zhao J, Mo H, Guo X, Wang Q, Xu D, Hou Y, et al. Red blood cell distribution width as a related factor of pulmonary arterial hypertension in patients with systemic sclerosis. Clin Rheumatol. 2018;37:979-985 pubmed publisher
    ..3%) and 14 of dcSSc (23.0%) subjects. Patients with higher RDW values were more likely to be men with high anti-u1RNP titers and PAH. A significant correlation was found between RDW and high-sensitivity C-reactive protein (p?=?0...
  42. Woppmann A, Will C, Kornstädt U, Zuo P, Manley J, Luhrmann R. Identification of an snRNP-associated kinase activity that phosphorylates arginine/serine rich domains typical of splicing factors. Nucleic Acids Res. 1993;21:2815-22 pubmed
    ..A potential general role for this enzyme in the phosphorylation of splicing factors and its consequences for pre-mRNA splicing regulation are discussed. ..
  43. Davis M, Hatzubai A, Andersen J, Ben Shushan E, Fisher G, Yaron A, et al. Pseudosubstrate regulation of the SCF(beta-TrCP) ubiquitin ligase by hnRNP-U. Genes Dev. 2002;16:439-51 pubmed
    ..Our study points to a novel regulatory mechanism, which secures the localization, stability, substrate binding threshold, and efficacy of a specific protein-ubiquitin ligase. ..
  44. 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. ..
  45. Greidinger E, Foecking M, Schäfermeyer K, Bailey C, Primm S, Lee D, et al. T cell immunity in connective tissue disease patients targets the RNA binding domain of the U1-70kDa small nuclear ribonucleoprotein. J Immunol. 2002;169:3429-37 pubmed
    ..The T cell autoimmune response to U1-70kDa appears to have less diversity than is seen in the humoral response; and therefore, may be a favorable target for therapeutic intervention. ..
  46. Tzang B, Chen D, Tsai C, Chiang S, Lin T, Hsu T. Human parvovirus B19 nonstructural protein NS1 enhanced the expression of cleavage of 70 kDa U1-snRNP autoantigen. J Biomed Sci. 2010;17:40 pubmed publisher
    ..These findings suggested crucial association of B19-NS1 in development of autoimmunity by inducing apoptosis and specific cleavage of 70 kDa U1-snRNP...
  47. Katsumata Y, Kawaguchi Y, Baba S, Hattori S, Tahara K, Ito K, et al. Serum antibodies against the 70k polypeptides of the U1 ribonucleoprotein complex are associated with psychiatric syndromes in systemic lupus erythematosus: a retrospective study. Mod Rheumatol. 2013;23:71-80 pubmed publisher
    ..The anti-U1-70k antibodies might be involved in the pathological mechanisms of psychiatric syndromes in SLE. ..
  48. Fong Y, Zhou Q. Stimulatory effect of splicing factors on transcriptional elongation. Nature. 2001;414:929-33 pubmed
    ..Because the TAT-SF1-U snRNP complex also stimulates splicing in vitro, it may serve as a dual-function factor to couple transcription and splicing and to facilitate their reciprocal activation. ..
  49. Shin M, Kang Y, Lee N, Kim S, Kang K, Lazova R, et al. U1-small nuclear ribonucleoprotein activates the NLRP3 inflammasome in human monocytes. J Immunol. 2012;188:4769-75 pubmed publisher
    ..Our findings indicate that endogenous RNA-containing U1-snRNP could be a signal that activates the NLRP3 inflammasome in autoimmune diseases like systemic lupus erythematosus where anti-U1-snRNP Abs are present. ..
  50. Kondo Y, Oubridge C, van Roon A, Nagai K. Crystal structure of human U1 snRNP, a small nuclear ribonucleoprotein particle, reveals the mechanism of 5' splice site recognition. elife. 2015;4: pubmed publisher
  51. Spritz R, Strunk K, Surowy C, Mohrenweiser H. Human U1-70K ribonucleoprotein antigen gene: organization, nucleotide sequence, and mapping to locus 19q13.3. Genomics. 1990;8:371-9 pubmed
    ..This extraordinary conservation at the nucleotide sequence level suggests that alternative splicing of this exon serves an important function, perhaps in regulating the production of functional U1-70K protein. ..
  52. Greidinger E, Foecking M, Magee J, Wilson L, Ranatunga S, Ortmann R, et al. A major B cell epitope present on the apoptotic but not the intact form of the U1-70-kDa ribonucleoprotein autoantigen. J Immunol. 2004;172:709-16 pubmed
    ..Immunization of mice against this epitope induces an immune response with features seen in human anti-70k autoimmune disease. ..
  53. Haudek K, Voss P, Wang J, Patterson R. A 10S galectin-3-U1 snRNP complex assembles into active spliceosomes. Nucleic Acids Res. 2016;44:6391-7 pubmed publisher
    ..These results indicate that the galectin-3-U1 snRNP-pre-mRNA ternary complex is a functional E complex and that U1 snRNP is required to assemble galectin-3 onto an active spliceosome. ..
  54. Macri C, Wang F, Tasset I, Schall N, Page N, Briand J, et al. Modulation of deregulated chaperone-mediated autophagy by a phosphopeptide. Autophagy. 2015;11:472-86 pubmed publisher
    The P140 peptide, a 21-mer linear peptide (sequence 131-151) generated from the spliceosomal SNRNP70/U1-70K protein, contains a phosphoserine residue at position 140...
  55. 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. ..
  56. Yu Y, Reed R. FUS functions in coupling transcription to splicing by mediating an interaction between RNAP II and U1 snRNP. Proc Natl Acad Sci U S A. 2015;112:8608-13 pubmed publisher
    ..Together, our data lead to a model that FUS functions in coupling transcription to splicing via mediating an interaction between RNAP II and U1 snRNP. ..
  57. Shav Tal Y, Cohen M, Lapter S, Dye B, Patton J, Vandekerckhove J, et al. Nuclear relocalization of the pre-mRNA splicing factor PSF during apoptosis involves hyperphosphorylation, masking of antigenic epitopes, and changes in protein interactions. Mol Biol Cell. 2001;12:2328-40 pubmed
    ..In apoptotic cells, PSF dissociated from PTB and bound new partners, including the U1--70K and SR proteins and therefore may acquire new functions. ..
  58. So B, Wan L, Zhang Z, Li P, Babiash E, Duan J, et al. A U1 snRNP-specific assembly pathway reveals the SMN complex as a versatile hub for RNP exchange. Nat Struct Mol Biol. 2016;23:225-30 pubmed publisher
    ..We propose that SMN-Gemin2 is a versatile hub for RNP exchange that functions broadly in RNA metabolism. ..
  59. Spiluttini B, Gu B, Belagal P, Smirnova A, Nguyen V, HEBERT C, et al. Splicing-independent recruitment of U1 snRNP to a transcription unit in living cells. J Cell Sci. 2010;123:2085-93 pubmed publisher
    ..By contrast, U1 snRNA, the U1-70K (also known as snRNP70) U1-associated protein as well as the ASF/SF2 (also known as SFRS1) serine/arginine-rich (SR) protein were ..
  60. Shin C, Feng Y, Manley J. Dephosphorylated SRp38 acts as a splicing repressor in response to heat shock. Nature. 2004;427:553-8 pubmed
    ..SRp38 thus plays a crucial role in cell survival under stress conditions by inhibiting the splicing machinery. ..
  61. Dumortier H, Klein Gunnewiek J, Roussel J, van Aarssen Y, Briand J, van Venrooij W, et al. At least three linear regions but not the zinc-finger domain of U1C protein are exposed at the surface of the protein in solution and on the human spliceosomal U1 snRNP particle. Nucleic Acids Res. 1998;26:5486-91 pubmed
    ..The N-terminal region of U1C, which is functional in U1 snRNP assembly, is apparently not located at the surface of the U1 snRNP particle. ..
  62. Stejskalova E, Stanek D. The splicing factor U1-70K interacts with the SMN complex and is required for nuclear gem integrity. J Cell Sci. 2014;127:3909-15 pubmed publisher
    ..These findings show that U1-70K is an SMN-complex-associating protein, and they suggest a new function for U1-70K in the formation of nuclear gems. ..
  63. Northemann W, Berg H, Stahnke G, Walter M, Hunt N, Fenning S. Identification of an inhibitory element within the human 68-kDa (U1) ribonucleoprotein antigen. Protein Expr Purif. 1995;6:748-56 pubmed
    ..After excision of the inhibitory sequence X a dramatic increase in the production of recombinant human P68 antigen was observed. ..
  64. Weber G, Trowitzsch S, Kastner B, Luhrmann R, Wahl M. Functional organization of the Sm core in the crystal structure of human U1 snRNP. EMBO J. 2010;29:4172-84 pubmed publisher
    ..The intricate, multi-layered interplay of proteins and RNA rationalizes the hierarchical assembly of U snRNPs in vitro and in vivo. ..
  65. Diner I, Hales C, Bishof I, Rabenold L, Duong D, Yi H, et al. Aggregation properties of the small nuclear ribonucleoprotein U1-70K in Alzheimer disease. J Biol Chem. 2014;289:35296-313 pubmed publisher
    ..Our results support a hypothesis that aberrant forms of U1-70K in AD can directly sequester soluble forms of U1-70K into insoluble aggregates. ..
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    ..A sequence comparison with two recently cloned RNA binding proteins revealed homology with one region in the U1 RNP 70K protein. This domain may also be responsible for RNA binding. ..
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    ..serology (viral capsid antigen, IgG, IgM) and autoantibodies by immunoprecipitation and ELISA (IgG and IgM class, U1RNP/Sm, U1-70 k, P peptide, rheumatoid factor, dsDNA, beta2-glycoprotein I)...
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    ..The results suggest a model for ESE function in which the SRm160/300 splicing coactivator promotes critical interactions between ESE-bound "activators" and the snRNP machinery of the spliceosome. ..
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    ..Research in this field could improve the understanding of the mechanisms leading to anti-U1-70 kDa autoimmunity, with potential consequences on prevention. ..
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    ..These results suggest a putative role of FCP1 CTD-phosphatase in linking the transcription elongation with the splicing process. ..
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    ..We discuss these results with respect to the differential protein-protein interactions that must occur during constitutive and activated splicing. ..
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    ..Together, our data suggest that IL-32α associates with PLZF and PKCɛ, and then inhibits PLZF sumoylation, resulting in suppression of the transcriptional activity of PLZF. ..
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    ..Knowledge of the precise epitopes of U1-70K autoantibodies may provide insight into the mechanisms of development of anti-RNP, identify potential clinical biomarkers and inform ongoing clinical trails of peptide-based therapeutics. ..
  76. Awasthi S, Alwine J. Association of polyadenylation cleavage factor I with U1 snRNP. RNA. 2003;9:1400-9 pubmed
    ..In addition, the 25-kD CF Im subunit binds specifically to the 70K protein of U1 snRNP (U1 70K). This binding may account for the CF Im/U1 snRNP interaction. During these studies we found that mAb 2.73 (mAb 2...
  77. 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. ..
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    ..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. ..