mRNA前体剪接因子PRPF8抗体
规格：1mg/1ml
英文名： PRPF8
别名： 220 kDa U5 snRNP specific protein; 220 kDa U5 snRNP-specific protein; Apoptosis regulated protein 1; Apoptosis regulated protein 2; HPRP8; p220; Pre mRNA processing factor 8; Pre mRNA-processing facto
分子量： 274kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PRPF8
交叉反应：Human, Mouse, Cat, Rhesus monkey, Gorilla, Orangutan, Xenopus tropicalis
细胞定位：细胞核
mRNA前体剪接因子PRPF8抗体产品介绍：background: Pre-mRNA splicing occurs in 2 sequential transesterification steps. The protein encoded by this gene is a component of both U2- and U12-dependent spliceosomes, and found to be essential for the catalytic step II in pre-mRNA splicing process. It contains several WD repeats, which function in protein-protein interactions. This protein has a sequence similarity to yeast Prp8 protein. This gene is a candidate gene for autosomal dominant retinitis pigmentosa. [provided by RefSeq, Jul 2008] Function: Functions as a scaffold that mediates the ordered assembly of spliceosomal proteins and snRNAs. Required for the assembly of the U4/U6-U5 tri-snRNP complex. Functions as scaffold that positions spliceosomal U2, U5 and U6 snRNAs at splice sites on pre-mRNA substrates, so that splicing can occur. Interacts with both the 5' and the 3' splice site. Subunit: Part of the U5 snRNP complex. Component of the U4/U6-U5 tri-snRNP complex composed of the U4, U6 and U5 snRNAs and at least PRPF3, PRPF4, PRPF6, PRPF8, PRPF31, SNRNP200, TXNL4A, SNRNP40, DDX23, CD2BP2, PPIH, NHP2L1,mRNA前体剪接因子PRPF8抗体 EFTUD2, SART1 and USP39. Component of the U5.U4atac/U6atac snRNP complexes in U12-dependent spliceosomes. Found in a mRNA splicing-dependent exon junction complex (EJC) with SRRM1. Interacts with U5 snRNP proteins SNRP116 and SNRNP40. Interacts with EFTUD2 and SNRNP200. Interacts (via the MPN (JAB/Mov34) domain) with PRPF3 ('Lys-63'-linked polyubiquitinated); may stabilize the U4/U6-U5 tri-snRNP complex. Subcellular Location: Nucleus speckle. Tissue Specificity: Widely expressed. Post-translational modifications: Phosphorylated upon DNA damage, probably by ATM or ATR. DISEASE: Defects in PRPF8 are the cause of retinitis pigmentosa type 13 (RP13) [MIM:600059]. RP leads to mRNA前体剪接因子PRPF8抗体degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. RP13 inheritance is autosomal dominant. Similarity: Contains 1 MPN (JAB/Mov34) domain. Gene ID: 10594 Database links: Entrez Gene: 10594 Human Entrez Gene: 192159 Mouse Entrez Gene: 287530 Rat Omim: 607300 Human SwissProt: Q6P2Q9 Human SwissProt: Q99PV0 Mouse Unigene: 181368 Human Unigene: 3757 Mouse Unigene: 106432 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
mRNA前体剪接因子PRPF8抗体产品应用：ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 神经生物学 干细胞 结合蛋白 表观遗传学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid