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.
Applications:Suitable for use in Western Blot, Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemistry: 1:50-1:200Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：50ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid PBS, 0.1% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of PRPF3. Species Crossreactivity: Human, mouse, rat
