| 1. |
- Lankester, Arjan C, et al.
(författare)
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Hematopoietic cell transplantation in severe combined immunodeficiency: The SCETIDE 2006-2014 European cohort.
- 2022
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Ingår i: The Journal of allergy and clinical immunology. - : Elsevier BV. - 1097-6825 .- 0091-6749. ; 149:5
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Tidskriftsartikel (refereegranskat)abstract
- Hematopoietic stem cell transplantation (HSCT) represents a curative treatment for patients with severe combined immunodeficiency (SCID), a group of monogenic immune disorders with an otherwise fatal outcome.We performed a comprehensive multicenter analysis of genotype-specific HSCT outcome, including detailed analysis of immune reconstitution (IR) and the predictive value for clinical outcome.HSCT outcome was studied in 338 patients with genetically confirmed SCID who underwent transplantation in 2006-2014 and who were registered in the SCETIDE registry. In a representative subgroup of 152 patients, data on IR and long-term clinical outcome were analyzed.Two-year OS was similar with matched family and unrelated donors and better than mismatched donor HSCT (P<.001). The 2-year event-free survival (EFS) was similar in matched and mismatched unrelated donor and less favorable in mismatched related donor (MMRD) HSCT (P< .001). Genetic subgroups did not differ in 2-year OS (P= .1) and EFS (P=.073). In multivariate analysis, pretransplantation infections and use of MMRDs were associated with less favorable OS and EFS. With a median follow-up of 6.2 years (range, 2.0-11.8 years), 73 of 152 patients in the IR cohort were alive and well without Ig dependency. IL-2 receptor gamma chain/Janus kinase 3/IL-7 receptor-deficient SCID, myeloablative conditioning, matched donor HSCT, and naive CD4 T lymphocytes >0.5×10e3/μL at+1 year were identified as independent predictors of favorable clinical and immunologic outcome.Recent advances in HSCT in SCID patients have resulted in improved OS and EFS in all genotypes and donor types. To achieve a favorable long-term outcome, treatment strategies should aim for optimal naive CD4 T lymphocyte regeneration.
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| 2. |
- Teilum, Kaare, et al.
(författare)
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Transient structural distortion of metal-free Cu/Zn superoxide dismutase triggers aberrant oligomerization.
- 2009
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Ingår i: Proceedings of the National Academy of Sciences. - : Proceedings of the National Academy of Sciences. - 1091-6490 .- 0027-8424. ; 106:43, s. 18273-18278
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Tidskriftsartikel (refereegranskat)abstract
- Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease linked to the misfolding of Cu/Zn superoxide dismutase (SOD1). ALS-related defects in SOD1 result in a gain of toxic function that coincides with aberrant oligomerization. The structural events triggering oligomerization have remained enigmatic, however, as is the case in other protein-misfolding diseases. Here, we target the critical conformational change that defines the earliest step toward aggregation. Using nuclear spin relaxation dispersion experiments, we identified a short-lived (0.4 ms) and weakly populated (0.7%) conformation of metal-depleted SOD1 that triggers aberrant oligomerization. This excited state emanates from the folded ground state and is suppressed by metal binding, but is present in both the disulfide-oxidized and disulfide-reduced forms of the protein. Our results pinpoint a perturbed region of the excited-state structure that forms intermolecular contacts in the earliest nonnative dimer/oligomer. The conformational transition that triggers oligomerization is a common feature of WT SOD1 and ALS-associated mutants that have widely different physicochemical properties. But compared with WT SOD1, the mutants have enhanced structural distortions in their excited states, and in some cases slightly higher excited-state populations and lower kinetic barriers, implying increased susceptibility to oligomerization. Our results provide a unified picture that highlights both (i) a common denominator among different SOD1 variants that may explain why diverse mutations cause the same disease, and (ii) a structural basis that may aid in understanding how different mutations affect disease propensity and progression.
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| 3. |
- Aggestam, Karin, et al.
(författare)
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Peacebuilding in the Middle East
- 2019
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Ingår i: Routledge Handbook on Middle East Security. - 9781138749894 ; , s. 141-153
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Bokkapitel (refereegranskat)
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| 4. |
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| 5. |
- Andreasson, Jakob, 1975, et al.
(författare)
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Electron-lattice interactions in the perovskite LaFe0.5Cr0.5O3 characterized by optical spectroscopy and LDA plus U calculations
- 2009
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Ingår i: Physical Review B. Condensed Matter and Materials Physics. - 1098-0121 .- 1550-235X .- 2469-9950 .- 2469-9969. ; 80:7, s. 075103-
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Tidskriftsartikel (refereegranskat)abstract
- We use resonance Raman scattering (incident photon energies between 1.8 and 4.13 eV), LDA+U calculations, spectroscopic ellipsometry, and oblique IR reflectivity to characterize the strong electron-phonon interactions in the disordered perovskite LaFe0.5Cr0.5O3. When the photon energy coincides with a Cr to Fe Mott-Hubbard transfer gap around 2.4 eV the electron-phonon interaction is manifested by a Franck-Condon effect with exceptional first-and higher order scattering of a local oxygen breathing mode. At higher incident energies we observe a superposition of Franck-Condon scattering and Frohlich interaction induced infrared active longitudinal optical two-phonon scattering activated mainly by O to Fe charge transfer. Our results establish LaFe0.5Cr0.5O3 as a model compound for research on electron-phonon interactions in strongly correlated complex systems and show that Franck-Condon scattering in complex solids is not limited to Jahn-Teller active compounds.
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| 6. |
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| 7. |
- Baaz, Mikael, 1966, et al.
(författare)
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Defining and Analyzing “Resistance”: Possible Entrances to the Study of Subversive Practices
- 2016
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Ingår i: Alternatives: Global, Local, Political. - THOUSAND OAKS, CA 91320 USA : SAGE Publications. - 0304-3754 .- 2163-3150. ; 41:3, s. 137-153
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Tidskriftsartikel (refereegranskat)abstract
- This article explores the meaning of “resistance” and suggests a new path for “resistance studies,” which is an emerging and interdisciplinary field of the social sciences that is still relatively fragmented and heterogeneous. Resistance has often been connected with antisocial attitudes, destructiveness, reactionary or revolutionary ideologies, unusual and sudden explosions of violence, and emotional outbursts. However, we wish to add to this conceptualization by arguing that resistance also has the potential to be productive, plural and fluid, and integrated into everyday social life. The first major part of the article is devoted to discuss existing understandings of resistance with the aim of seeking to capture distinctive features and boundaries of this social phenomenon. Among other things, we will explore resistance in relation to other key concepts and related research fields. We then, in the article’s second major part, propose a number of analytical categories and possible entrances aiming at inspire more in-depth studies of resistance.
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| 8. |
- Baaz, Mikael, 1966, et al.
(författare)
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The ABC of resistance: towards a new analytical framework
- 2023
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Ingår i: Journal of Political Power. - 2158-379X .- 2158-3803. ; 16:1
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Tidskriftsartikel (refereegranskat)abstract
- Two significant developments – (1) the rapidly changing world order, and (2) significant gaps in current social science scholarship – call for a further exploration of resistance theories. In this paper, we identify some of the gaps and inconsistencies within the current bulk of research, and seek to contribute to the understanding of resistance, its applications and complexity. In short, this paper discusses three interacting and supporting forms of resistance, including various overlaps and interlinkages between them, which together constitute what we would like to call the ABC of resistance; that is, avoidance resistance, breaking resistance, and constructive resistance.
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