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- 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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- Amare, Azmeraw T, et al.
(författare)
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Association of polygenic score and the involvement of cholinergic and glutamatergic pathways with lithium treatment response in patients with bipolar disorder.
- 2023
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Ingår i: Molecular psychiatry. - 1476-5578. ; 28, s. 5251-5261
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Tidskriftsartikel (refereegranskat)abstract
- Lithium is regarded as the first-line treatment for bipolar disorder (BD), a severe and disabling mental healthdisorder that affects about 1% of the population worldwide. Nevertheless, lithium is not consistently effective, with only 30% of patients showing a favorable response to treatment. To provide personalized treatment options for bipolar patients, it is essential to identify prediction biomarkers such as polygenic scores. In this study, we developed a polygenic score for lithium treatment response (Li+PGS) in patients with BD. To gain further insights into lithium's possible molecular mechanism of action, we performed a genome-wide gene-based analysis. Using polygenic score modeling, via methods incorporating Bayesian regression and continuous shrinkage priors, Li+PGS was developed in the International Consortium of Lithium Genetics cohort (ConLi+Gen: N=2367) and replicated in the combined PsyCourse (N=89) and BipoLife (N=102) studies. The associations of Li+PGS and lithium treatment response - defined in a continuous ALDA scale and a categorical outcome (good response vs. poor response) were tested using regression models, each adjusted for the covariates: age, sex, and the first four genetic principal components. Statistical significance was determined at P<0.05. Li+PGS was positively associated with lithium treatment response in the ConLi+Gen cohort, in both the categorical (P=9.8×10-12, R2=1.9%) and continuous (P=6.4×10-9, R2=2.6%) outcomes. Compared to bipolar patients in the 1st decile of the risk distribution, individuals in the 10th decile had 3.47-fold (95%CI: 2.22-5.47) higher odds of responding favorably to lithium. The results were replicated in the independent cohorts for the categorical treatment outcome (P=3.9×10-4, R2=0.9%), but not for the continuous outcome (P=0.13). Gene-based analyses revealed 36 candidate genes that are enriched in biological pathways controlled by glutamate and acetylcholine. Li+PGS may be useful in the development of pharmacogenomic testing strategies by enabling a classification of bipolar patients according to their response to treatment.
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- Joshi, Deep C., et al.
(författare)
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2D crystal structure and anisotropic magnetism of GdAu6.75−xAl0.5+x (x ≈ 0.54)
- 2022
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Ingår i: Scientific Reports. - : Springer Science and Business Media LLC. - 2045-2322. ; 12
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Tidskriftsartikel (refereegranskat)abstract
- Exploration of the gold-rich part of the ternary Gd–Au–Al system afforded the intermetallic compound GdAu6.75−xAl0.5+x (x ≈ 0.54) which was structurally characterized by single crystal X-ray diffraction (Pnma, a = 18.7847(4) Å, b = 23.8208(5) Å, c = 5.3010(1) Å). GdAu6.75−xAl0.5+x crystallizes in a previously unknown structure type featuring layers of Gd2(Au, Al)29 and Gd2(Au, Al)28 clusters which are arranged as in a close-packing parallel to the ac plane. The Gd substructure corresponds to slightly corrugated 36 nets (dGd–Gd = 5.30–5.41 Å) which are stacked on top of each other along the b direction with alternating short (5.4, 5.6 Å, within layers) and long distances (6.4 Å, between layers). The title compound has been discussed with respect to a quasicrystal approximant (1/1 AC) GdAu5.3Al in the same system. The magnetic properties of GdAu6.75−xAl0.5+x were found to be reminiscent to those of some ternary ACs, with sharp peaks in the temperature dependent magnetization, and metamagnetic-like transitions. The material becomes antiferromagnetic below 25 K; magnetometry results suggest that the antiferromagnetic state is composed of ferromagnetic ac planes, coupled antiferromagnetically along the b direction.
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| 5. |
- Xiao, Tianxiao, et al.
(författare)
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Autonomous self-healing hybrid energy harvester based on the combination of triboelectric nanogenerator and quantum dot solar cell
- 2024
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Ingår i: Nano Energy. - : Elsevier BV. - 2211-2855 .- 2211-3282. ; 125
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Tidskriftsartikel (refereegranskat)abstract
- Realization of multi-source energy harvesting with one single device would maximize power output. Thus, it is emerging as a promising strategy towards renewable energy generation and has attracted worldwide attention in the past decades. Capable of capturing mechanical energy that is ubiquitous in the ambient environment, triboelectric nanogenerator (TENG) has been considered a novel yet effective source towards next-generation energy harvesting. In this work, a flexible hybrid energy harvester (HEH) is developed via the rational integration of autonomous self-healing TENG and high bending-stable lead sulfide quantum dot (PbS QD) solar cell, enabling independent electricity generation by two different mechanisms. The single-electrode mode TENG component with self-healing is realized by a polydimethylsiloxane/Triton X-100 (PDMS/TX100) mixture as the dielectric layer and the shared gold (Au) electrode, which generates 0.39 µA of output current (Iout), 24.6 V of output voltages (Vout), 15.4 nC of transfer charges (Qsc), and 7.80 mW m−2 of output power peak density. The thin-film solar cell component is based on a PbS QD layer as the light absorber with a planar structure fabricated under low-cost and compatible conditions, achieving 22.8 mA cm−2 of short-circuit current density (Jsc) and 4.92% of power conversion efficiency (PCE). As a proof of concept, an electronic watch is successfully powered by harnessing ambient mechanical and solar energy with a hybridized energy cell. This approach will offer more opportunities to construct a versatile platform towards remote monitoring and smart home systems.
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- Zheng, Daniel J., et al.
(författare)
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Linker-Dependent Stability of Metal-Hydroxide Organic Frameworks for Oxygen Evolution
- 2023
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Ingår i: Chemistry of Materials. - : American Chemical Society (ACS). - 0897-4756 .- 1520-5002. ; 35:13, s. 5017-5031
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Tidskriftsartikel (refereegranskat)abstract
- Metal-organic frameworks (MOFs) are periodic organic-inorganicmaterials that have garnered considerable attention for electrocatalyticapplications due to their wide tunability. Metal-hydroxide organicframeworks (MHOFs), a subset of MOFs that combine layered metal hydroxideswith organic ligands of various & pi;-& pi; stacking energy,have shown promising catalytic functions, such as for the oxygen evolutionreaction (OER). The long-term electrochemical stability of these materialsfor the OER is unfortunately not well understood, which is criticalto design practical devices. In this study, we investigated how Ni-basedMHOFs composed of two linkers with different & pi;-& pi;interaction strength (terephthalate; L1 and azobenzene-4,4 & PRIME;-dicarboxylate;L4) change as a function of cycle number and potential for the OER.All MHOFs tested showed significant increases in the number of electrochemicallyactive Ni sites and OER activity when cycled. MHOFs constructed usingthe linkers with stronger & pi;-& pi; stacking energy (L4)were observed to remain intact in bulk with only near-surface transformationsto NiOOH2-x -like phases, whereasMHOFs with linkers of weaker & pi;-& pi; stacking energy(L1) showed complete reconstruction to NiOOH2-x -like phases. This was confirmed using X-ray diffraction,X-ray absorption spectroscopy, and electron microscopy. Further, insitu characterization using Raman and UV-vis revealed thatthe presence of stable linkers within the MHOF structure suppressesthe Ni2+/Ni(3+& delta;)+ redox process. We furtheridentify NiOOH2-x as the OER activephase, while the MHOF phase serves as a precatalyst. We further proposea detailed mechanism for the phase transformation, which providesvaluable insights into the future challenges for the design of bothstable and catalytically active MOF-based materials for water oxidation.
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