Product Name	Anti-PRPF8/RBITC
Chinese Name	罗丹明（RBITC）标记的mRNA前体剪接因子PRPF8抗体
Alias	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 factor 8, S. cerevisiae, homolog of; Pre-mRNA-processing-splicing factor 8; Precursor mRNA processing protein; PRP8; PRP8 homolog; PRP8 pre mRNA processing factor 8 homolog; PRP8_HUMAN; PRPC8; Prpf8; Retinitis pigmentosa 13 (autosomal dominant); RP13; SNRNP220; Splicing factor Prp8; U5 snRNP specific protein (220 kD), ortholog of S. cerevisiae Prp8p; U5 snRNP specific protein.
Research Area	Cell biology  Neurobiology  Stem cells  Binding protein  Epigenetics
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Human, Mouse, Rhesus monkey, Gorilla, Orangutan, Xenopus tropicalis)
Applications	ICC=1:50-200 IF=1:50-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Molecular weight	274kDa
Form	Lyophilized or Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human PRPF8
Lsotype	IgG
Purification	affinity purified by Protein A
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Product Detail	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, 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 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. 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.

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