PRPF8, Blocking Peptide

Pre-mRNA-processing-splicing factor 8; 220 kDa U5 snRNP-specific protein; PRP8 homolog; Splicing factor Prp8; p220; PRPF8; PRPC8

For Research Use Only. Not for use in diagnostic procedures.

The synthetic peptide sequence is selected from aa 2239-2253 of HUMAN PRPF8

Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Small volumes of PRPF8 blocking peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

273,600 Da

pre-mRNA processing factor 8

pre-mRNA-processing-splicing factor 8

Pre-mRNA-processing-splicing factor 8

PRPC8  [Similar Products]

PRP8_HUMAN

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]

PRPF8: Central component of the spliceosome, which may play a role in aligning the pre-mRNA 5'- and 3'-exons for ligation. Interacts with U5 snRNA, and with pre-mRNA 5'-splice sites in B spliceosomes and 3'-splice sites in C spliceosomes. Defects in PRPF8 are the cause of retinitis pigmentosa type 13 (RP13). 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.

Protein type: Spliceosome; RNA-binding; RNA splicing

Chromosomal Location of Human Ortholog: 17p13.3

Cellular Component: membrane; nucleoplasm; nucleus; snRNP U5

Molecular Function: protein binding; second spliceosomal transesterification activity; U1 snRNA binding; U2 snRNA binding; U5 snRNA binding; U6 snRNA binding

Biological Process: assembly of spliceosomal tri-snRNP; mRNA processing; nuclear mRNA splicing, via spliceosome; RNA splicing; RNA splicing, via transesterification reactions

Disease: Retinitis Pigmentosa 13

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