PRPF3 抗体 (42-N): sc-101130

PRPF3 Antibody (42-N) is an IgG₃ κ mouse monoclonal PRPF3 antibody (also designated PRPF3 antibody) suitable for the detection of the PRPF3 protein of mouse, rat and human origin by WB, IP and ELISA. PRPF3 Antibody (42-N) is available as the non-conjugated anti-PRPF3 antibody. PRPF3 (PRP3 pre-mRNA processing factor 3 homolog), also known as RP18, PRP3, Prp3p, HPRP3 or HPRP3P, is an evolutionarily conserved protein involved in pre-mRNA splicing and functions as a component of the U4/U6.U5 tri-snRNP (small nuclear ribonucleoprotein) complex. Ubiquitously expressed with predominant expression in retina, blood, kidney and liver, PRPF3 localizes to nuclear speckles and is phosphorylated in vitro. PRPF3 directly interacts with PRPF4 and is present in the inactive spliceosome but is not found in the catalytically active spliceosome. Mutations in the gene encoding PRPF3 result in autosomal dominant retinitis pigmentosa type 18 (RP18), which leads to photoreceptor cell degeneration. RP18 patients initially exhibit a loss of their midperipheral visual field as well as night vision blindness. The disease eventually progresses to the loss of far peripheral visual field and finally the loss of central vision. This suggests that PRPF3 is a key player in the pre-mRNA splicing of photoreceptor-specific genes.

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References:

1. Mutations in HPRP3, a third member of pre-mRNA splicing factor genes, implicated in autosomal dominant retinitis pigmentosa.  |  Chakarova, CF., et al. 2002. Hum Mol Genet. 11: 87-92. PMID: 11773002
2. Central region of the human splicing factor Hprp3p interacts with Hprp4p.  |  Gonzalez-Santos, JM., et al. 2002. J Biol Chem. 277: 23764-72. PMID: 11971898
3. Mutations in splicing factor PRPF3, causing retinal degeneration, form detrimental aggregates in photoreceptor cells.  |  Comitato, A., et al. 2007. Hum Mol Genet. 16: 1699-707. PMID: 17517693
4. Mutation in the splicing factor Hprp3p linked to retinitis pigmentosa impairs interactions within the U4/U6 snRNP complex.  |  Gonzalez-Santos, JM., et al. 2008. Hum Mol Genet. 17: 225-39. PMID: 17932117
5. Hypoxia-regulated components of the U4/U6.U5 tri-small nuclear riboprotein complex: possible role in autosomal dominant retinitis pigmentosa.  |  Schmidt-Kastner, R., et al. 2008. Mol Vis. 14: 125-35. PMID: 18334927
6. The human U4/U6 snRNP contains 60 and 90kD proteins that are structurally homologous to the yeast splicing factors Prp4p and Prp3p.  |  Lauber, J., et al. 1997. RNA. 3: 926-41. PMID: 9257651
7. Identification and characterization of human genes encoding Hprp3p and Hprp4p, interacting components of the spliceosome.  |  Wang, A., et al. 1997. Hum Mol Genet. 6: 2117-26. PMID: 9328476
8. A new cyclophilin and the human homologues of yeast Prp3 and Prp4 form a complex associated with U4/U6 snRNPs.  |  Horowitz, DS., et al. 1997. RNA. 3: 1374-87. PMID: 9404889