PRPF3 explained

U4/U6 small nuclear ribonucleoprotein Prp3 is a protein that in humans is encoded by the PRPF3 gene.^[1]

Function

See also: U4 spliceosomal RNA and U6 spliceosomal RNA. The removal of introns from nuclear pre-mRNAs occurs on complexes called spliceosomes, which are made up of 4 small nuclear ribonucleoprotein (snRNP) particles and an undefined number of transiently associated splicing factors. PRPF3 is one of several proteins that associate with U4 and U6 snRNPs.[supplied by OMIM]^[1]

Interactions

PRPF3 has been shown to interact with DVL3.^[2]

Further reading

• Maruyama K, Sugano S . Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides . Gene . 138 . 1–2 . 171–4 . Jan 1994 . 8125298 . 10.1016/0378-1119(94)90802-8 .
• Xu SY, Schwartz M, Rosenberg T, Gal A . A ninth locus (RP18) for autosomal dominant retinitis pigmentosa maps in the pericentromeric region of chromosome 1 . Human Molecular Genetics . 5 . 8 . 1193–7 . Aug 1996 . 8842740 . 10.1093/hmg/5.8.1193 . free .
• Wang A, Forman-Kay J, Luo Y, Luo M, Chow YH, Plumb J, Friesen JD, Tsui LC, Heng HH, Woolford JL, Hu J . Identification and characterization of human genes encoding Hprp3p and Hprp4p, interacting components of the spliceosome . Human Molecular Genetics . 6 . 12 . 2117–26 . Nov 1997 . 9328476 . 10.1093/hmg/6.12.2117 . free .
• Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, Suyama A, Sugano S . Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library . Gene . 200 . 1–2 . 149–56 . Oct 1997 . 9373149 . 10.1016/S0378-1119(97)00411-3 .
• Horowitz DS, Kobayashi R, Krainer AR . A new cyclophilin and the human homologues of yeast Prp3 and Prp4 form a complex associated with U4/U6 snRNPs . RNA . 3 . 12 . 1374–87 . Dec 1997 . 9404889 . 1369579 .
• Heng HH, Wang A, Hu J . Mapping of the human HPRP3 and HPRP4 genes encoding U4/U6-associated splicing factors to chromosomes 1q21.1 and 9q31-q33 . Genomics . 48 . 2 . 273–5 . Mar 1998 . 9521884 . 10.1006/geno.1997.5181 .
• Xu SY, Rosenberg T, Gal A . Refined genetic mapping of autosomal dominant retinitis pigmentosa locus RP18 reduces the critical region to 2 cM between D1S442 and D1S2858 on chromosome 1q . Human Genetics . 102 . 4 . 493–4 . Apr 1998 . 9600251 . 10.1007/s004390050728 . 39815355 .
• Reidt U, Reuter K, Achsel T, Ingelfinger D, Lührmann R, Ficner R . Crystal structure of the human U4/U6 small nuclear ribonucleoprotein particle-specific SnuCyp-20, a nuclear cyclophilin . The Journal of Biological Chemistry . 275 . 11 . 7439–42 . Mar 2000 . 10713041 . 10.1074/jbc.275.11.7439 . 20261807 . free .
• Chakarova CF, Hims MM, Bolz H, Abu-Safieh L, Patel RJ, Papaioannou MG, Inglehearn CF, Keen TJ, Willis C, Moore AT, Rosenberg T, Webster AR, Bird AC, Gal A, Hunt D, Vithana EN, Bhattacharya SS . Mutations in HPRP3, a third member of pre-mRNA splicing factor genes, implicated in autosomal dominant retinitis pigmentosa . Human Molecular Genetics . 11 . 1 . 87–92 . Jan 2002 . 11773002 . 10.1093/hmg/11.1.87 . free .
• Horowitz DS, Lee EJ, Mabon SA, Misteli T . A cyclophilin functions in pre-mRNA splicing . The EMBO Journal . 21 . 3 . 470–80 . Feb 2002 . 11823439 . 125845 . 10.1093/emboj/21.3.470 .
• Gonzalez-Santos JM, Wang A, Jones J, Ushida C, Liu J, Hu J . Central region of the human splicing factor Hprp3p interacts with Hprp4p . The Journal of Biological Chemistry . 277 . 26 . 23764–72 . Jun 2002 . 11971898 . 10.1074/jbc.M111461200 . free .
• Nottrott S, Urlaub H, Lührmann R . Hierarchical, clustered protein interactions with U4/U6 snRNA: a biochemical role for U4/U6 proteins . The EMBO Journal . 21 . 20 . 5527–38 . Oct 2002 . 12374753 . 129076 . 10.1093/emboj/cdf544 .
• Ajuh P, Chusainow J, Ryder U, Lamond AI . A novel function for human factor C1 (HCF-1), a host protein required for herpes simplex virus infection, in pre-mRNA splicing . The EMBO Journal . 21 . 23 . 6590–602 . Dec 2002 . 12456665 . 136956 . 10.1093/emboj/cdf652 .
• Martínez-Gimeno M, Gamundi MJ, Hernan I, Maseras M, Millá E, Ayuso C, García-Sandoval B, Beneyto M, Vilela C, Baiget M, Antiñolo G, Carballo M . Mutations in the pre-mRNA splicing-factor genes PRPF3, PRPF8, and PRPF31 in Spanish families with autosomal dominant retinitis pigmentosa . Investigative Ophthalmology & Visual Science . 44 . 5 . 2171–7 . May 2003 . 12714658 . 10.1167/iovs.02-0871 . free .
• Reidt U, Wahl MC, Fasshauer D, Horowitz DS, Lührmann R, Ficner R . Crystal structure of a complex between human spliceosomal cyclophilin H and a U4/U6 snRNP-60K peptide . Journal of Molecular Biology . 331 . 1 . 45–56 . Aug 2003 . 12875835 . 10.1016/S0022-2836(03)00684-3 . 11858/00-001M-0000-0012-F238-B . free .
• Wada Y, Itabashi T, Sato H, Tamai M . Clinical features of a Japanese family with autosomal dominant retinitis pigmentosa associated with a Thr494Met mutation in the HPRP3 gene . Graefe's Archive for Clinical and Experimental Ophthalmology = Albrecht von Graefes Archiv für Klinische und Experimentelle Ophthalmologie . 242 . 11 . 956–61 . Nov 2004 . 15085354 . 10.1007/s00417-004-0923-x . 22773384 .
• Stanĕk D, Neugebauer KM . Detection of snRNP assembly intermediates in Cajal bodies by fluorescence resonance energy transfer . The Journal of Cell Biology . 166 . 7 . 1015–25 . Sep 2004 . 15452143 . 2172029 . 10.1083/jcb.200405160 .
• Maita H, Kitaura H, Ariga H, Iguchi-Ariga SM . Association of PAP-1 and Prp3p, the products of causative genes of dominant retinitis pigmentosa, in the tri-snRNP complex . Experimental Cell Research . 302 . 1 . 61–8 . Jan 2005 . 15541726 . 10.1016/j.yexcr.2004.08.022 .

External links

• GeneReviews/NIH/NCBI/UW entry on Retinitis Pigmentosa Overview

Notes and References

1. Web site: Entrez Gene: PRPF3 PRP3 pre-mRNA processing factor 3 homolog (S. cerevisiae).
2. Rual JF, Venkatesan K, Hao T, Hirozane-Kishikawa T, Dricot A, Li N, Berriz GF, Gibbons FD, Dreze M, Ayivi-Guedehoussou N, Klitgord N, Simon C, Boxem M, Milstein S, Rosenberg J, Goldberg DS, Zhang LV, Wong SL, Franklin G, Li S, Albala JS, Lim J, Fraughton C, Llamosas E, Cevik S, Bex C, Lamesch P, Sikorski RS, Vandenhaute J, Zoghbi HY, Smolyar A, Bosak S, Sequerra R, Doucette-Stamm L, Cusick ME, Hill DE, Roth FP, Vidal M . 6 . Towards a proteome-scale map of the human protein-protein interaction network . Nature . 437 . 7062 . 1173–8 . Oct 2005 . 16189514 . 10.1038/nature04209 . 2005Natur.437.1173R . 4427026 .