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U5 small nuclear ribonucleoprotein 200 kDa helicase

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RNA helicase that plays an essential role in pre-mRNA splicing as component of the U5 snRNP and U4/U6-U5 tri-snRNP complexes. Involved in spliceosome assembly, activation and disassembly. Mediates changes in the dynamic network of RNA-RNA interactions in the spliceosome. Catalyzes the ATP-dependent unwinding of U4/U6 RNA duplices, an essential step in the assembly of a catalytically active spliceosome.

Below are the list of possible U5 small nuclear ribonucleoprotein 200 kDa helicase products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.
 

U5 small nuclear ribonucleoprotein 200 kDa helicase

 U5 small nuclear ribonucleoprotein 200 kDa helicase ELISA Kit
 U5 small nuclear ribonucleoprotein 200 kDa helicase Recombinant
 U5 small nuclear ribonucleoprotein 200 kDa helicase Antibody
Also known as U5 small nuclear ribonucleoprotein 200 kDa helicase (Activating signal cointegrator 1 complex subunit 3-like 1) (BRR2 homolog) (U5 snRNP-specific 200 kDa protein) (U5-200KD).
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 retinopath
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ies. 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: EC 3.6.4.13; Helicase; RNA processing; RNA splicing; RNA-binding; Spliceosome

Chromosomal Location of Human Ortholog: 2q11.2

Cellular Component: membrane; nucleoplasm; nucleus; snRNP U5; spliceosome

Molecular Function: ATP-dependent helicase activity; ATP-dependent RNA helicase activity; identical protein binding; protein binding

Biological Process: cis assembly of pre-catalytic spliceosome; nuclear mRNA splicing, via spliceosome; osteoblast differentiation

Disease: Retinitis Pigmentosa 33
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