Small Nuclear Ribonucleoprotein 200kDa (SNRNP200)

[Edit]

ASCC3L1; U5-200KD; HELIC2; BRR2; U5 SnRNP Specific Protein,200 KD; Activating Signal Cointegrator 1 Complex Subunit 3-Like 1

Small Nuclear Ribonucleoprotein 200kDa (SNRNP200)
Pre-mRNA splicing is catalyzed by the spliceosome, a complex of specialized RNA and protein subunits that removes introns from a transcribed pre-mRNA segment. The spliceosome consists of small nuclear RNA proteins (snRNPs) U1, U2, U4, U5 and U6, together with approximately 80 conserved proteins. U5 snRNP contains nine specific proteins.
SNRNP200 encodes one of the U5 snRNP-specific proteins. This protein belongs to the DEXH-box family of putative RNA helicases. It is a core component of U4/U6-U5 snRNPs and appears to catalyze an ATP-dependent unwinding of U4/U6 RNA duplices. Mutations in this gene cause autosomal-dominant retinitis pigmentosa. Alternatively spliced transcript variants encoding different isoforms have been found, but the full-length nature of these variants has not been determined.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Small Nuclear Ribonucleoprotein 200kDa (SNRNP200) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Small Nuclear Ribonucleoprotein 200kDa (SNRNP200) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Small Nuclear Ribonucleoprotein 200kDa (SNRNP200) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Small Nuclear Ribonucleoprotein 200kDa (SNRNP200) CLIA Kit Customized Service Offer
n/a ELISA Kit for Small Nuclear Ribonucleoprotein 200kDa (SNRNP200) ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Small Nuclear Ribonucleoprotein 200kDa (SNRNP200) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Small Nuclear Ribonucleoprotein 200kDa (SNRNP200) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Small Nuclear Ribonucleoprotein 200kDa (SNRNP200) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Small Nuclear Ribonucleoprotein 200kDa (SNRNP200) CLIA Kit Customized Service Offer
n/a ELISA Kit for Small Nuclear Ribonucleoprotein 200kDa (SNRNP200) ELISA Kit Customized Service Offer
  1. "The network of protein-protein interactions within the human U4/U6.U5 tri-snRNP."RNA 12:1418-1430(2006) [PubMed] [Europe PMC] [Abstract]
  2. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  3. "Prediction of the coding sequences of unidentified human genes. XI. The complete sequences of 100 new cDNA clones from brain which code for large proteins in vitro."DNA Res. 5:277-286(1998) [PubMed] [Europe PMC] [Abstract]
  4. "Construction of expression-ready cDNA clones for KIAA genes: manual curation of 330 KIAA cDNA clones."DNA Res. 9:99-106(2002) [PubMed] [Europe PMC] [Abstract]
  5. "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. 15:4001-4015(1996) [PubMed] [Europe PMC] [Abstract]
  6. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  7. "The human U5-200kD DEXH-box protein unwinds U4/U6 RNA duplices in vitro."Proc. Natl. Acad. Sci. U.S.A. 95:4188-4192(1998) [PubMed] [Europe PMC] [Abstract]
  8. "Purification and characterization of native spliceosomes suitable for three-dimensional structural analysis."RNA 8:426-439(2002) [PubMed] [Europe PMC] [Abstract]
  9. "A proteomic study of SUMO-2 target proteins."J. Biol. Chem. 279:33791-33798(2004) [PubMed] [Europe PMC] [Abstract]
  10. "Global, in vivo, and site-specific phosphorylation dynamics in signaling networks."Cell 127:635-648(2006) [PubMed] [Europe PMC] [Abstract]
  11. "Phosphorylation analysis of primary human T lymphocytes using sequential IMAC and titanium oxide enrichment."J. Proteome Res. 7:5167-5176(2008) [PubMed] [Europe PMC] [Abstract]
  12. "Kinase-selective enrichment enables quantitative phosphoproteomics of the kinome across the cell cycle."Mol. Cell 31:438-448(2008) [PubMed] [Europe PMC] [Abstract]
  13. "A quantitative atlas of mitotic phosphorylation."Proc. Natl. Acad. Sci. U.S.A. 105:10762-10767(2008) [PubMed] [Europe PMC] [Abstract]
  14. "Large-scale phosphoproteome analysis of human liver tissue by enrichment and fractionation of phosphopeptides with strong anion exchange chromatography."Proteomics 8:1346-1361(2008) [PubMed] [Europe PMC] [Abstract]
  15. "Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach."Anal. Chem. 81:4493-4501(2009) [PubMed] [Europe PMC] [Abstract]
  16. "Quantitative phosphoproteomic analysis of T cell receptor signaling reveals system-wide modulation of protein-protein interactions."Sci. Signal. 2:RA46-RA46(2009) [PubMed] [Europe PMC] [Abstract]
  17. "Lysine acetylation targets protein complexes and co-regulates major cellular functions."Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract]
  18. "Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis."Sci. Signal. 3:RA3-RA3(2010) [PubMed] [Europe PMC] [Abstract]
  19. "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
  20. "System-wide temporal characterization of the proteome and phosphoproteome of human embryonic stem cell differentiation."Sci. Signal. 4:RS3-RS3(2011) [PubMed] [Europe PMC] [Abstract]
  21. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  22. "Crystal structure of Q9P172/SEC63 from Homo sapiens."Submitted (FEB-2009) to the PDB data bank
  23. "Structural basis for functional cooperation between tandem helicase cassettes in Brr2-mediated remodeling of the spliceosome."Proc. Natl. Acad. Sci. U.S.A. 109:17418-17423(2012) [PubMed] [Europe PMC] [Abstract]
  24. "The consensus coding sequences of human breast and colorectal cancers." Science 314:268-274(2006) [PubMed] [Europe PMC] [Abstract]
  25. "Autosomal-dominant retinitis pigmentosa caused by a mutation in SNRNP200, a gene required for unwinding of U4/U6 snRNAs."Am. J. Hum. Genet. 85:617-627(2009) [PubMed] [Europe PMC] [Abstract]
  26. "Mutations in ASCC3L1 on 2q11.2 are associated with autosomal dominant retinitis pigmentosa in a Chinese family."Invest. Ophthalmol. Vis. Sci. 51:1036-1043(2010) [PubMed] [Europe PMC] [Abstract]
  27. "Next generation sequencing of pooled samples reveals new SNRNP200 mutations associated with retinitis pigmentosa."Hum. Mutat. 32:E2246-E2258(2011) [PubMed] [Europe PMC] [Abstract]
  28. "A novel missense SNRNP200 mutation associated with autosomal dominant retinitis pigmentosa in a Chinese family."PLoS ONE 7:E45464-E45464(2012) [PubMed] [Europe PMC] [Abstract]
  29. "Contribution of SNRNP200 sequence variations to retinitis pigmentosa."Eye 27:1204-1213(2013) [PubMed] [Europe PMC] [Abstract]
  30. "Mutations in the small nuclear riboprotein 200 kDa gene (SNRNP200) cause 1.6% of autosomal dominant retinitis pigmentosa."Mol. Vis. 19:2407-2417(2013) [PubMed] [Europe PMC] [Abstract]