Human U5 small nuclear ribonucleoprotein 200 kDa helicase, SNRNP200 ELISA Kit | SNRNP200 elisa kit
Human U5 small nuclear ribonucleoprotein 200 kDa helicase, SNRNP200 ELISA Kit
Principle of the Assay: This kit is an Enzyme-Linked Immunosorbent Assay (ELISA). The plate has been pre-coated with Human SNRNP200 antibody. SNRNP200 present in the sample is added and binds to antibodies coated on the wells. And then biotinylated Human SNRNP200 Antibody is added and binds to SNRNP200 in the sample. Then Streptavidin-HRP is added and binds to the Biotinylated SNRNP200 antibody. After incubation unbound Streptavidin-HRP is washed away during a washing step. Substrate solution is then added and color develops in proportion to the amount of Human SNRNP200. The reaction is terminated by addition of acidic stop solution and absorbance is measured at 450 nm.
NCBI and Uniprot Product Information
NCBI Description
Pre-mRNA splicing is catalyzed by the spliceosome, a complex of specialized RNA and protein subunits that removes introns from a transcribed pre-mRNA segment. The spliceosome consists of small nuclear RNA proteins (snRNPs) U1, U2, U4, U5 and U6, together with approximately 80 conserved proteins. U5 snRNP contains nine specific proteins. This gene encodes one of the U5 snRNP-specific proteins. This protein belongs to the DEXH-box family of putative RNA helicases. It is a core component of U4/U6-U5 snRNPs and appears to catalyze an ATP-dependent unwinding of U4/U6 RNA duplices. Mutations in this gene cause autosomal-dominant retinitis pigmentosa. Alternatively spliced transcript variants encoding different isoforms have been found, but the full-length nature of these variants has not been determined. [provided by RefSeq, Mar 2010]
Uniprot Description
U5-200kD: Putative RNA helicase involved in the second step of RNA splicing. May promote one or more conformational changes in the dynamic network of RNA-RNA interactions in the spliceosome. Appears to catalyze an ATP-dependent unwinding of U4/U6 RNA duplices. Defects in SNRNP200 are the cause of retinitis pigmentosa type 33 (RP33). It is a retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. 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. Belongs to the helicase family. SKI2 subfamily. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: RNA splicing; Spliceosome; RNA-binding; Helicase; EC 3.6.4.13; RNA processing
Chromosomal Location of Human Ortholog: 2q11.2
Cellular Component: nucleoplasm; spliceosome; membrane; snRNP U5; nucleus
Molecular Function: identical protein binding; protein binding; ATP-dependent helicase activity; ATP-dependent RNA helicase activity; ATP binding
Biological Process: osteoblast differentiation; nuclear mRNA splicing, via spliceosome; cis assembly of pre-catalytic spliceosome; RNA splicing; gene expression
Disease: Retinitis Pigmentosa 33
Research Articles on SNRNP200
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Product Notes
The Human SNRNP200 snrnp200 (Catalog #AAA1607214) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA1607214 ELISA Kit recognizes Human SNRNP200. It is sometimes possible for the material contained within the vial of "U5 small nuclear ribonucleoprotein 200 kDa helicase, SNRNP200, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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