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- Byrne, M., et al.
(författare)
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Expression of the neuropeptide SALMFamide-1 during regeneration of the seastar radial nerve cord following arm autotomy
- 2019
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Ingår i: Proceedings of the Royal Society B-Biological Sciences. - : The Royal Society. - 0962-8452 .- 1471-2954. ; 286:1901
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Tidskriftsartikel (refereegranskat)abstract
- Arm loss through a separation at a specialized autotomy plane in echinoderms is inextricably linked to regeneration, but the link between these phenomena is poorly explored. We investigated nervous system regeneration post-autotomy in the asteriid seastar Coscinasterias muricata, focusing on the reorganization of the radial nerve cord (RNC) into the ectoneural neuroepithelium and neuropile, and the hyponeural region, using antibodies to the seastar-specific neuropeptide SALMFamide-1 (S1). Parallel changes in the associated haemal and coelomic vessels were also examined. A new arm bud appeared in 3-5 days with regeneration over three weeks. At the nerve stump and in the RNC immediately behind, the haemal sinus/hyponeural coelomic compartments enlarged into a hypertrophied space filled with migratory cells that appear to be involved in wound healing and regeneration. The haemal and coelomic compartments provided a conduit for these cells to gain rapid access to the regeneration site. An increase in the number of glia-like cells indicates the importance of these cells in regeneration. Proximal to the autotomy plane, the original RNC exhibited Wallerian-type degeneration, as seen in disorganized axons and enlarged S1-positive varicosities. The imperative to regrow lost arms quickly is reflected in the efficiency of regeneration from the autotomy plane facilitated by the rapid appearance of progenitor-like migratory cells. In parallel to its specialization for defensive arm detachment, the autotomy plane appears to be adapted to promote regeneration. This highlights the importance of examining autotomy-induced regeneration in seastars as a model system to study nervous system regeneration in deuterostomes and the mechanisms involved with the massive migration of stem-like cells to facilitate rapid recovery.
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- Mercuri, E., et al.
(författare)
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Nusinersen versus Sham Control in Later-Onset Spinal Muscular Atrophy
- 2018
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Ingår i: New England Journal of Medicine. - 0028-4793. ; 378:7, s. 625-635
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND Nusinersen is an antisense oligonucleotide drug that modulates pre-messenger RNA splicing of the survival motor neuron 2 (SMN2) gene. It has been developed for the treatment of spinal muscular atrophy (SMA). METHODS We conducted a multicenter, double-blind, sham-controlled, phase 3 trial of nusinersen in 126 children with SMA who had symptom onset after 6 months of age. The children were randomly assigned, in a 2: 1 ratio, to undergo intrathecal administration of nusinersen at a dose of 12 mg (nusinersen group) or a sham procedure (control group) on days 1, 29, 85, and 274. The primary end point was the least-squares mean change from baseline in the Hammersmith Functional Motor Scale-Expanded (HFMSE) score at 15 months of treatment; HFMSE scores range from 0 to 66, with higher scores indicating better motor function. Secondary end points included the percentage of children with a clinically meaningful increase from baseline in the HFMSE score (>= 3 points), an outcome that indicates improvement in at least two motor skills. RESULTS In the prespecified interim analysis, there was a least-squares mean increase from baseline to month 15 in the HFMSE score in the nusinersen group (by 4.0 points) and a least-squares mean decrease in the control group (by -1.9 points), with a significant between-group difference favoring nusinersen (least-squares mean difference in change, 5.9 points; 95% confidence interval, 3.7 to 8.1; P< 0.001). This result prompted early termination of the trial. Results of the final analysis were consistent with results of the interim analysis. In the final analysis, 57% of the children in the nusinersen group as compared with 26% in the control group had an increase from baseline to month 15 in the HFMSE score of at least 3 points (P< 0.001), and the overall incidence of adverse events was similar in the nusinersen group and the control group (93% and 100%, respectively). CONCLUSIONS Among children with later-onset SMA, those who received nusinersen had significant and clinically meaningful improvement in motor function as compared with those in the control group. (Funded by Biogen and Ionis Pharmaceuticals; CHERISH ClinicalTrials. gov number, NCT02292537.)
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- Riva, M., et al.
(författare)
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High-Priority Prototype Testing in Support of System-Level Design Development of the ITER Radial Neutron Camera
- 2018
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Ingår i: IEEE Transactions on Plasma Science. - : IEEE-INST ELECTRICAL ELECTRONICS ENGINEERS INC. - 0093-3813 .- 1939-9375. ; 46:5, s. 1291-1297
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Tidskriftsartikel (refereegranskat)abstract
- This paper describes the high-priority testing activities supporting the ITER radial neutron camera (RNC) design, performed by a consortium of European institutes within a framework contract placed by fusion for energy, the ITER European Domestic Agency. The main role of the RNC is to measure the uncollided 14- and 2.5-MeV neutrons from deuterium-tritium and deuterium-deuterium fusion reactions through an array of flux monitors/spectrometers located in collimated lines of sight viewing the plasma through the ITER equatorial port plug #1. The line-integrated neutron fluxes will be used to evaluate, through reconstruction techniques, the radial profile of the neutrons emitted per unit time and volume (neutron emissivity) and, therefore, the neutron yield and the alpha particles' birth profile. The activity of high-priority testing is dedicated to the preparation and the design of experimental test environment, the conduction of appropriate tests and reporting of test results for the high-priority prototypes, clarifying or verifying the expected key function and system behavior, and enhancing learning on specific issues (potential showstoppers).
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