Gene Symbol: SNRPD1
Description: small nuclear ribonucleoprotein D1 polypeptide
Alias: HsT2456, SMD1, SNRPD, Sm-D1, small nuclear ribonucleoprotein Sm D1, Sm-D autoantigen, small nuclear ribonucleoprotein D1 polypeptide 16kDa pseudogene, snRNP core protein D1
Species: human
Products:     SNRPD1

Top Publications

  1. Mammoto A, Sasaki T, Kim Y, Takai Y. Physical and functional interaction of rabphilin-11 with mammalian Sec13 protein. Implication in vesicle trafficking. J Biol Chem. 2000;275:13167-70 pubmed
    ..These results indicate that the rabphilin-11-mSec13p interaction is implicated in vesicle trafficking. ..
  2. 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. ..
  3. Liu Q, Fischer U, Wang F, Dreyfuss G. The spinal muscular atrophy disease gene product, SMN, and its associated protein SIP1 are in a complex with spliceosomal snRNP proteins. Cell. 1997;90:1013-21 pubmed
    ..These findings suggest a role for SMN and SIP1 in spliceosomal snRNP biogenesis and function and provide a likely molecular mechanism for the cause of SMA. ..
  4. Sleeman J, Lamond A. Newly assembled snRNPs associate with coiled bodies before speckles, suggesting a nuclear snRNP maturation pathway. Curr Biol. 1999;9:1065-74 pubmed
    ..These include speckles, coiled bodies and nucleoli, in addition to a diffuse nucleoplasmic compartment. The reasons for snRNP accumulation in these different structures are unclear...
  5. Kambach C, Walke S, Young R, Avis J, de La Fortelle E, Raker V, et al. Crystal structures of two Sm protein complexes and their implications for the assembly of the spliceosomal snRNPs. Cell. 1999;96:375-87 pubmed
    ..The crystal structures suggest that the seven Sm proteins could form a closed ring and the snRNAs may be bound in the positively charged central hole. ..
  6. Charroux B, Pellizzoni L, Perkinson R, Shevchenko A, Mann M, Dreyfuss G. Gemin3: A novel DEAD box protein that interacts with SMN, the spinal muscular atrophy gene product, and is a component of gems. J Cell Biol. 1999;147:1181-94 pubmed
    ..The presence of a DEAD box motif in Gemin3 suggests that it may provide the catalytic activity that plays a critical role in the function of the SMN complex on RNPs. ..
  7. 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. ..
  8. 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. ..
  9. Zhang R, So B, Li P, Yong J, Glisovic T, Wan L, et al. Structure of a key intermediate of the SMN complex reveals Gemin2's crucial function in snRNP assembly. Cell. 2011;146:384-95 pubmed publisher
    ..Here, we describe the 2.5 Å crystal structure of Gemin2 bound to SmD1/D2/F/E/G pentamer and SMN's Gemin2-binding domain, a key assembly intermediate...

More Information


  1. Zhao Z, Ren J, Dai C, Kannapell C, Wang H, Gaskin F, et al. Nature of T cell epitopes in lupus antigens and HLA-DR determines autoantibody initiation and diversification. Ann Rheum Dis. 2019;78:380-390 pubmed publisher
    ..T cell epitopes (T-epitopes) of SmD1 (SmD) were mapped by T-T hybridomas generated from DR3+AE0 mice immunised with SmD and with ..
  2. Deng H, Li Z, Dai G, Yuan Y, Ning Y, Shangguan J, et al. Isolation of new polymorphic microsatellite markers from the marbled rockfish Sebastiscus marmoratus. Genet Mol Res. 2015;14:758-62 pubmed publisher
    ..751. The observed and expected heterozygosity levels were 0.0333-0.9667 and 0.0328-0.7675, respectively. Two loci, Smd1-112 and Smd2-80, deviated from Hardy-Weinberg equilibrium...
  3. Schwer B, Roth A, Shuman S. Will the circle be unbroken: specific mutations in the yeast Sm protein ring expose a requirement for assembly factor Brr1, a homolog of Gemin2. RNA. 2017;23:420-430 pubmed publisher
    ..mutagenesis in Saccharomyces cerevisiae, we gained new insights into structure-function relationships of the SmD1 and SmD2 subunits...
  4. Gomez V, Wasserman M. Interactions between Giardia duodenalis Sm proteins and their association with spliceosomal snRNAs. Parasitol Res. 2017;116:617-626 pubmed publisher
    ..Immunoprecipitation with anti-HA revealed that the SmD3 protein is associated with the proteins SmB, SmD1, SmD2, SmE and SmF in vivo...
  5. Elvira Matelot E, Bardou F, Ariel F, Jauvion V, Bouteiller N, Le Masson I, et al. The Nuclear Ribonucleoprotein SmD1 Interplays with Splicing, RNA Quality Control, and Posttranscriptional Gene Silencing in Arabidopsis. Plant Cell. 2016;28:426-38 pubmed publisher
    ..thaliana mutant smd1b was identified in a genetic screen for PTGS deficiency, revealing the involvement of SmD1, a component of the Smith (Sm) complex, in PTGS...
  6. Shen W, Deng X, Zou W, Cheng F, Engelhardt J, Yan Z, et al. Identification and Functional Analysis of Novel Nonstructural Proteins of Human Bocavirus 1. J Virol. 2015;89:10097-109 pubmed publisher
    ..We constructed mutant pIHBoV1 infectious plasmids which harbor silent mutations (sm) smA1' and smD1' at the A1' and D1' splice sites, respectively...
  7. Li J, Leung A, Kondo Y, Oubridge C, Nagai K. Re-refinement of the spliceosomal U4 snRNP core-domain structure. Acta Crystallogr D Struct Biol. 2016;72:131-46 pubmed publisher
    ..The U4 Sm site-sequence AAUUUUU has been shown to bind to the seven Sm proteins SmF-SmE-SmG-SmD3-SmB-SmD1-SmD2 in an identical manner as the U1 Sm-site sequence AAUUUGU, except in SmD1 where the bound U replaces G...
  8. Kim Y, Lee J, Kim H, Lee M, Son M, Yoo C, et al. The unique spliceosome signature of human pluripotent stem cells is mediated by SNRPA1, SNRPD1, and PNN. Stem Cell Res. 2017;22:43-53 pubmed publisher
    ..Among them, SNRPA1, SNRPD1, and PNN were significantly up-regulated during the early stage of reprogramming, identified as hub genes by ..
  9. Yang H, Zhang X, Fu Q. Evaluating Anti-SmD1-amino-acid 83-119 Peptide Reactivity in Children with Systemic Lupus Erythematosus and Other Immunological Diseases. Chin Med J (Engl). 2016;129:2840-2844 pubmed publisher
    b>SmD1-amino-acid 83-119 peptide (SmD183-119) is the major epitope of Smith (Sm) antigen, which is specific for adult systemic lupus erythematosus (SLE)...
  10. Xiong X, Vogler G, Kurthkoti K, Samsonova A, Zhou R. SmD1 Modulates the miRNA Pathway Independently of Its Pre-mRNA Splicing Function. PLoS Genet. 2015;11:e1005475 pubmed publisher
    ..Here, we show that SmD1, a core component of the Drosophila small nuclear ribonucleoprotein particle (snRNP) implicated in splicing, is ..
  11. Hu C, Li M, Liu J, Qian J, Xu D, Zhang S, et al. Anti-SmD1 antibodies are associated with renal disorder, seizures, and pulmonary arterial hypertension in Chinese patients with active SLE. Sci Rep. 2017;7:7617 pubmed publisher
    ..In this study, we used a SmD1 polypeptide-based ELISA to determine anti-SmD1 antibody in 269 SLE, including100 naïve (had not been treated with ..
  12. Friesen W, Wyce A, Paushkin S, Abel L, Rappsilber J, Mann M, et al. A novel WD repeat protein component of the methylosome binds Sm proteins. J Biol Chem. 2002;277:8243-7 pubmed
  13. Azzouz T, Pillai R, Däpp C, Chari A, Meister G, Kambach C, et al. Toward an assembly line for U7 snRNPs: interactions of U7-specific Lsm proteins with PRMT5 and SMN complexes. J Biol Chem. 2005;280:34435-40 pubmed
    ..Furthermore, we present evidence for two separate binding sites in SMN for Sm/Lsm proteins. One recognizes Sm domains and the second one, the sDMA-modified RG-tails, which are present only in a subset of these proteins. ..
  14. Yong J, Wan L, Dreyfuss G. Why do cells need an assembly machine for RNA-protein complexes?. Trends Cell Biol. 2004;14:226-32 pubmed
    ..We discuss how this surveillance capacity of the SMN complex might ensure assembly of Sm cores only on the correct RNAs and prevent illicit, potentially deleterious assembly of Sm cores on random RNAs. ..
  15. Tian Q, Feetham M, Tao W, He X, Li L, Aebersold R, et al. Proteomic analysis identifies that 14-3-3zeta interacts with beta-catenin and facilitates its activation by Akt. Proc Natl Acad Sci U S A. 2004;101:15370-5 pubmed
    ..We propose that Akt phosphorylates beta-catenin, which results in 14-3-3zeta binding and stabilization of beta-catenin, and these interactions may be involved in stem cell development. ..
  16. 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
  17. Park J, Voss P, Grabski S, Wang J, Patterson R. Association of galectin-1 and galectin-3 with Gemin4 in complexes containing the SMN protein. Nucleic Acids Res. 2001;29:3595-602 pubmed
  18. Fury M, Zhang W, Christodoulopoulos I, Zieve G. Multiple protein: protein interactions between the snRNP common core proteins. Exp Cell Res. 1997;237:63-9 pubmed
    ..Other interactions were between proteins in the 6S and 20S particles or within the 20S particle itself. These interactions are likely to occur within the cytoplasmic snRNP core protein complexes and the mature snRNP particle. ..
  19. Friesen W, Paushkin S, Wyce A, Massenet S, Pesiridis G, Van Duyne G, et al. The methylosome, a 20S complex containing JBP1 and pICln, produces dimethylarginine-modified Sm proteins. Mol Cell Biol. 2001;21:8289-300 pubmed
    ..and directly to the symmetrical dimethylarginine (sDMA)-modified arginine- and glycine-rich (RG-rich) domains of SmD1 and SmD3...
  20. 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. ..
  21. 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. ..
  22. Leung A, Nagai K, Li J. Structure of the spliceosomal U4 snRNP core domain and its implication for snRNP biogenesis. Nature. 2011;473:536-9 pubmed publisher
    ..of the spliceosome, U1, U2, U4 and U5 small nuclear ribonucleoproteins (snRNPs), contain seven Sm proteins (SmB/B', SmD1, SmD2, SmD3, SmE, SmF and SmG) in common...
  23. 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. ..
  24. Mei Y, Wang P, Jiang C, Wang T, Chen L, Li Z, et al. Clinical and serological associations of anti-ribosomal P0 protein antibodies in systemic lupus erythematosus. Clin Rheumatol. 2018;37:703-707 pubmed publisher
    ..Anti-nucleosomes, anti-dsDNA, anti-Histones, anti-SmD1, and anti-U1snRNP were significantly associated with serum anti-Rib-P0 antibodies positivity in SLE patients (all P..
  25. 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. ..
  26. 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. ..
  27. Lennerz V, Fatho M, Gentilini C, Frye R, Lifke A, Ferel D, et al. The response of autologous T cells to a human melanoma is dominated by mutated neoantigens. Proc Natl Acad Sci U S A. 2005;102:16013-8 pubmed
    ..The mutations were found in the genes SIRT2, GPNMB, SNRP116, SNRPD1, and RBAF600. Peptides containing the mutated residues were presented by HLA-A(*)03011, -B(*)07021, and -B(*)3801...
  28. Pillai R, Grimmler M, Meister G, Will C, Luhrmann R, Fischer U, et al. Unique Sm core structure of U7 snRNPs: assembly by a specialized SMN complex and the role of a new component, Lsm11, in histone RNA processing. Genes Dev. 2003;17:2321-33 pubmed
    ..RNA 3' processing contains a structurally similar but biochemically unique Sm core in which two of these proteins, Sm D1 and D2, are replaced by Lsm10 and by another as yet unknown component...
  29. Iwai A, Hasumura Y, Nojima T, Takegami T. Hepatitis C virus nonstructural protein NS3 binds to Sm-D1, a small nuclear ribonucleoprotein associated with autoimmune disease. Microbiol Immunol. 2003;47:601-11 pubmed
    ..The localization of NS3 could change following interaction with Sm-D1 and affect the function of Sm-D1...
  30. Friesen W, Massenet S, Paushkin S, Wyce A, Dreyfuss G. SMN, the product of the spinal muscular atrophy gene, binds preferentially to dimethylarginine-containing protein targets. Mol Cell. 2001;7:1111-7 pubmed
    ..SMN binds the arginine- and glycine-rich (RG) domains of the snRNP proteins SmD1 and SmD3...
  31. Sun D, Ou Y, Hoch S. Analysis of genes for human snRNP Sm-D1 protein and identification of the promoter sequence which shows segmental homology to the promoters of Sm-E and U1 snRNA genes. Gene. 1997;189:245-54 pubmed
    ..They appear to be processed pseudogenes. The third gene, termed SNRPD1, shares 100% identity to the cDNA sequence including both 5'- and 3'-untranslated regions (UTR); it contains three ..
  32. Chari A, Golas M, Klingenhäger M, Neuenkirchen N, Sander B, Englbrecht C, et al. An assembly chaperone collaborates with the SMN complex to generate spliceosomal SnRNPs. Cell. 2008;135:497-509 pubmed publisher
    ..The mode of action of this combined chaperone/catalyst system is reminiscent of the mechanism employed by DNA clamp loaders. ..
  33. Quidville V, Alsafadi S, Goubar A, Commo F, Scott V, Pioche Durieu C, et al. Targeting the deregulated spliceosome core machinery in cancer cells triggers mTOR blockade and autophagy. Cancer Res. 2013;73:2247-58 pubmed publisher
    ..siRNA-mediated depletion of SmE (SNRPE) or SmD1 (SNRPD1) led to a marked reduction of cell viability in breast, lung, and melanoma cancer cell lines, whereas it had ..
  34. Lehmeier T, Foulaki K, Luhrmann R. Evidence for three distinct D proteins, which react differentially with anti-Sm autoantibodies, in the cores of the major snRNPs U1, U2, U4/U6 and U5. Nucleic Acids Res. 1990;18:6475-84 pubmed
    ..Whether the additional band represents a distinct common snRNP protein or a post-translationally modified version of G is not yet known. ..
  35. 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. ..
  36. Friesen W, Dreyfuss G. Specific sequences of the Sm and Sm-like (Lsm) proteins mediate their interaction with the spinal muscular atrophy disease gene product (SMN). J Biol Chem. 2000;275:26370-5 pubmed
    ..We show that unique carboxyl-terminal arginine- and glycine-rich domains comprising the last 29 amino acids of SmD1 and the last 32 amino acids of SmD3 are necessary and sufficient for SMN binding...
  37. Hirohata S, Sakuma Y, Yanagida T, Yoshio T. Association of cerebrospinal fluid anti-Sm antibodies with acute confusional state in systemic lupus erythematosus. Arthritis Res Ther. 2014;16:450 pubmed publisher
    ..More importantly, the data indicate that anti-Sm is yet another autoantibody with presumed neural toxicity, but might not be the last. ..
  38. Rokeach L, Haselby J, Hoch S. Molecular cloning of a cDNA encoding the human Sm-D autoantigen. Proc Natl Acad Sci U S A. 1988;85:4832-6 pubmed
    ..Moreover, this C terminus shows interesting features: (i) a good homology to protamines as expected for a nucleic acid binding protein and (ii) a striking similarity to a region in the Epstein-Barr nuclear antigen. ..
  39. Gubitz A, Mourelatos Z, Abel L, Rappsilber J, Mann M, Dreyfuss G. Gemin5, a novel WD repeat protein component of the SMN complex that binds Sm proteins. J Biol Chem. 2002;277:5631-6 pubmed
    ..Furthermore, Gemin5 interacts with several of the snRNP core proteins including SmB, SmD1, SmD2, SmD3, and SmE, suggesting that it participates in the activities of the SMN complex in snRNP assembly...
  40. 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. ..
  41. Tapia O, Lafarga V, Bengoechea R, Palanca A, Lafarga M, Berciano M. The SMN Tudor SIM-like domain is key to SmD1 and coilin interactions and to Cajal body biogenesis. J Cell Sci. 2014;127:939-46 pubmed publisher
    ..of SIM-like mutant constructs abolishes the interaction of SMN with the spliceosomal SmD1 (also known as SNRPD1), severely decreases SMN-coilin interaction and prevents CB assembly...
  42. Ma Y, Dostie J, Dreyfuss G, Van Duyne G. The Gemin6-Gemin7 heterodimer from the survival of motor neurons complex has an Sm protein-like structure. Structure. 2005;13:883-92 pubmed
  43. Renz M, Heim C, Bräunling O, Czichos A, Wieland C, Seelig H. Expression of the major human ribonucleoprotein (RNP) autoantigens in Escherichia coli and their use in an EIA for screening sera from patients with autoimmune diseases. Clin Chem. 1989;35:1861-3 pubmed