Recombinant Human U5 small nuclear ribonucleoprotein 200 kDa helicase (SNRNP200), partial

Code CSB-YP022319HU
MSDS
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Source Yeast
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Code CSB-EP022319HU
MSDS
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Source E.coli
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Code CSB-EP022319HU-B
MSDS
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Source E.coli
Conjugate Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
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Code CSB-BP022319HU
MSDS
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Source Baculovirus
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Code CSB-MP022319HU
MSDS
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Source Mammalian cell
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Product Details

Purity
>85% (SDS-PAGE)
Target Names
SNRNP200
Uniprot No.
Alternative Names
Activating signal cointegrator 1 complex subunit 3-like 1; ASCC3L1; BRR2; BRR2 homolog; HELIC2; RP33; SNRNP200; U5 small nuclear ribonucleoprotein 200 kDa helicase; U5 snRNP-specific 200 kDa protein; U5-200KD; U520_HUMAN
Species
Homo sapiens (Human)
Protein Length
Partial
Tag Info
Tag type will be determined during the manufacturing process.
The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
Form
Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
Buffer before Lyophilization
Tris/PBS-based buffer, 6% Trehalose, pH 8.0
Reconstitution
We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
Troubleshooting and FAQs
Storage Condition
Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life
The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Lead Time
Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
Notes
Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet
Please contact us to get it.

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Target Background

Function
Plays role in pre-mRNA splicing as core component of precatalytic, catalytic and postcatalytic spliceosomal 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.
Gene References into Functions
  1. This work identifies a novel immunoregulatory role of the spliceosomal SNRNP200 helicase as an RNA sensor and TBK1 adaptor for the activation of IRF3-mediated antiviral innate response PMID: 27454487
  2. Our data suggest that BRR2 is an important factor in 5'-splice-site recognition and that the retinitis pigmentosa linked mutations c.3260C>T (p.S1087L) and c.3269G>T (p.R1090L) affect this BRR2 function. PMID: 24302620
  3. Mutations in SNRNP200 cause 1.6% of autosomal dominant retinitis pigmentosa. PMID: 24319334
  4. we evaluated the mutation profile in 24 exons containing the hotspots in SNRNP200 among a cohort of southern Han Chinese retinitis pigmentosa patients and controls. A total of 18 novel variants were detected. PMID: 23887765
  5. differential human cell culture splicing and cell cycle defect models due to perturbed levels of human U5 snRNP associated U5-200kD RNA helicase PMID: 23637979
  6. A novel missense SNRNP200 mutation associated with autosomal dominant retinitis pigmentosa in a Chinese family. PMID: 23029027
  7. results reveal the structural and functional interplay between two helicase cassettes in a tandem superfamily 2 enzyme and point to several sites through which Brr2 activity may be regulated PMID: 23045696
  8. 4 new missense changes (p.R681C, p.R681H, p.V683L, p.Y689C) affecting highly conserved codons were identified in 6 unrelated individuals, indicating that the prevalence of SNRNP200-associated autosomal dominant retinitis pigmentosa is relatively high. PMID: 21618346
  9. A novel locus for RP33 has been assigned to chromosomal region 2cen-q12.1, which in a Chinese kindred is associated with a relatively late onset form of retinitis pigmentosa. PMID: 16612614
  10. The results provide strong evidence that mutations in ASCC3L1 have resulted in autosomal dominant retinitis pigmentosa in this Chinese family. PMID: 19710410
  11. Autosomal-dominant retinitis pigmentosa caused by a mutation in SNRNP200 is reported. PMID: 19878916

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Involvement in disease
Retinitis pigmentosa 33 (RP33)
Subcellular Location
Nucleus.
Protein Families
Helicase family, SKI2 subfamily
Tissue Specificity
Widely expressed.
Database Links

HGNC: 30859

OMIM: 601664

KEGG: hsa:23020

STRING: 9606.ENSP00000317123

UniGene: Hs.246112

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