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anti-Human SNRNP200 Antikörper:
anti-Mouse (Murine) SNRNP200 Antikörper:
anti-Rat (Rattus) SNRNP200 Antikörper:
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Biochemical and biophysical analyses revealed that an intrinsically disordered region of FBP21 (zeige WBP4 Antikörper) binds to an extended surface of the C-terminal Sec63 (zeige SEC63 Antikörper) unit of Brr2. Additional contacts in the C-terminal helicase cassette are required for allosteric inhibition of Brr2 helicase activity.
This work identifies a novel immunoregulatory role of the spliceosomal SNRNP200 helicase as an RNA sensor and TBK1 (zeige TBK1 Antikörper) adaptor for the activation of IRF3 (zeige IRF3 Antikörper)-mediated antiviral innate response
Results show that SNW1 (zeige SNW1 Antikörper) directly associates with EFTUD2 (zeige EFTUD2 Antikörper) and SNRNP200 and that disruption of SNW1 (zeige SNW1 Antikörper) association with these proteins promotes the apoptosis of breast cancer cells.
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.
Mutations in SNRNP200 cause 1.6% of autosomal dominant retinitis pigmentosa.
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.
differential human cell culture splicing and cell cycle defect models due to perturbed levels of human U5 snRNP (zeige LSM2 Antikörper) associated U5-200kD RNA helicase (zeige DDX46 Antikörper)
these data show how a Ski2 (zeige SKIV2L Antikörper)-like RNA helicase (zeige DDX46 Antikörper) Brr2 can be reversibly inhibited by a protein cofactor Prp8 (zeige PRPF8 Antikörper) that directly competes with RNA substrate binding.
A novel missense SNRNP200 mutation associated with autosomal dominant retinitis pigmentosa in a Chinese family.
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
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.
, U5 small nuclear ribonucleoprotein 200 kDa helicase
, U5 snRNP-specific 200 kDa protein
, activating signal cointegrator 1 complex subunit 3-like 1