antibody

Mouse monoclonal antibody Anti-Human PRPF8

MBS120210

0.1 mg

345 USD

monoclonal

mouse

nonconjugated

2834C1a

western blot, immunocytochemistry, flow cytometry, FACS

Antibody

Mouse monoclonal antibody Anti-Human PRPF8

PRPF8

Homo sapiens PRP8 pre-mRNA processing factor 8 homolog (yeast) (PRPF8); PRP8; RP13; HPRP8; PRPC8

Homo sapiens pre-mRNA processing factor 8 (PRPF8), mRNA; Pre-mRNA-processing-splicing factor 8; pre-mRNA-processing-splicing factor 8; 220 kDa U5 snRNP-specific protein; PRP8 homolog; PRP8 pre-mRNA processing factor 8 homolog; U5 snRNP-specific protein (220 kD), ortholog of S. cerevisiae Prp8p; apoptosis-regulated protein 1; apoptosis-regulated protein 2; p220; precursor mRNA processing protein; splicing factor Prp8; pre-mRNA processing factor 8; 220 kDa U5 snRNP-specific protein; PRP8 homolog; Splicing factor Prp8; p220

PRPF8

PRPF8; PRPF8; PRP8; RP13; HPRP8; PRPC8; SNRNP220

PRP8_HUMAN

Monoclonal

IgG1

2834C1a

Mouse

Human

7311

100 ug/ml (1.0 ml)

Dot Blot (DB), Immunocytochemistry (ICC), Western Blot (WB), Flow Cytometry (FC/FACS)

Preparation: This antibody was purified using protein G column chromatography from culture supernatant of hybridoma cultured in a medium containing bovine IgG-depleted (approximately 95%) fetal bovine serum.

Sterility: Filtered through a 0.22 um membrane.

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. [NCBI Entrez Gene Summary]

91208425

NP_006436.3

NM_006445

273,600 Da

Gene Expression Pathway (105937); Processing Of Capped Intron-Containing Pre-mRNA Pathway (160950); Spliceosome Pathway (125136); Spliceosome Pathway (124832); Spliceosome, 35S U5-snRNP Pathway (413438); Spliceosome, 35S U5-snRNP Pathway (890602); Spliceosome, U4/U6.U5 Tri-snRNP Pathway (413445); Spliceosome, U4/U6.U5 Tri-snRNP Pathway (890601); MRNA Splicing Pathway (105951); MRNA Splicing - Major Pathway (105952)

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 splicing; RNA-binding. Chromosomal Location of Human Ortholog: 17p13.3. Cellular Component: nucleoplasm; membrane; snRNP U5; nuclear speck; nucleus. Molecular Function: U5 snRNA binding; protein binding; U6 snRNA binding. Biological Process: RNA splicing, via transesterification reactions; nuclear mRNA splicing, via spliceosome; assembly of spliceosomal tri-snRNP; RNA splicing; gene expression; mRNA processing. Disease: Retinitis Pigmentosa 13

0.1 mg

345 USD