The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Protein concentration is above or equal to 0.05 mg/ml.
Previously labelled as PRPF8.
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Preparation and Storage
Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.31% Glutathione, 0.79% Tris HCl
220 kDa U5 snRNP specific protein
220 kDa U5 snRNP-specific protein
Apoptosis regulated protein 1
Apoptosis regulated protein 2
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 pre mRNA processing factor 8 homolog
Retinitis pigmentosa 13 (autosomal dominant)
Splicing factor Prp8
U5 snRNP specific protein
U5 snRNP specific protein (220 kD), ortholog of S. cerevisiae Prp8p
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.
Involvement in 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.
Contains 1 MPN (JAB/Mov34) domain.
The MPN domain has structural similarity with viral ribonucleases and RNase H, but unlike RNases, it does not bind any metal ions.
Phosphorylated upon DNA damage, probably by ATM or ATR.