| 2. |
- Dewan, Ramita, et al.
(författare)
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Pathogenic Huntingtin Repeat Expansions in Patients with Frontotemporal Dementia and Amyotrophic Lateral Sclerosis.
- 2021
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Ingår i: Neuron. - : Elsevier BV. - 1097-4199 .- 0896-6273. ; 109:3, s. 448-460.e4
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Tidskriftsartikel (refereegranskat)abstract
- We examined the role of repeat expansions in the pathogenesis of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) by analyzing whole-genome sequence data from 2,442 FTD/ALS patients, 2,599 Lewy body dementia (LBD) patients, and 3,158 neurologically healthy subjects. Pathogenic expansions (range, 40-64 CAG repeats) in the huntingtin (HTT) gene were found in three (0.12%) patients diagnosed with pure FTD/ALS syndromes but were not present in the LBD or healthy cohorts. We replicated our findings in an independent collection of 3,674 FTD/ALS patients. Postmortem evaluations of two patients revealed the classical TDP-43 pathology of FTD/ALS, as well as huntingtin-positive, ubiquitin-positive aggregates in the frontal cortex. The neostriatal atrophy that pathologically defines Huntington's disease was absent in both cases. Our findings reveal an etiological relationship between HTT repeat expansions and FTD/ALS syndromes and indicate that genetic screening of FTD/ALS patients for HTT repeat expansions should be considered.
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| 3. |
- Banijamali, S. M., et al.
(författare)
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Effect of Ce Addition on the Tribological Behavior of ZK60 Mg-Alloy
- 2021
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Ingår i: Metals and Materials International. - : Springer. - 1598-9623 .- 2005-4149. ; 27:8, s. 2732-2742
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Tidskriftsartikel (refereegranskat)abstract
- The present work aims to study the tribological behavior of an extruded ZK60 alloy in the presence of Ce; in a previous study, among ZK60 alloys with different Ce addition rates, an alloy with 3 wt% of Ce was found to exhibit the most promising mechanical (e.g., hardness and strengths) properties, while its wear behavior remained unknown. The results of microstructural examinations by optical and electron microscopes show that Ce addition reduces the grain size from 6.1 to 2.0 μm. Besides, in addition to the precipitates already distributed in the base alloy (Mg7Zn3), Ce could promote the formation of a new precipitate (MgZn2Ce), increasing the total fraction of the precipitates. These microstructural evolutions enhance the strengths of the studied ZK60 alloy, as the yield and tensile strengths increase from 212 to 308 MPa and from 297 to 354 MPa, respectively. A pin on disc tribometer was employed to study the wear behavior of the developed alloy under different normal loads (5, 20, 40, and 60 N). The results show that the base and Ce-added alloys exhibit almost a similar frictional behavior, while the wear resistance of the Ce-added alloy is higher within the load ranges applied: (i) in low load conditions (5 and 20 N), where the abrasive wear is the active mechanism, the precipitates in the Ce-added alloy could enhance the wear resistance. (ii) Under the load of 40 N, oxidative wear is also an operative wear mechanism, leading to a sharp increase in the wear rate of the alloys. In this condition, Ce could provide a protective oxide layer, which could improve the wear resistance of the alloy. (iii) At a load of 60 N, both studied alloys exhibit a similar wear rate due to a severe oxidation condition. Therefore, beyond this loading condition, the microstructural evolutions (e.g., change in precipitation behavior) caused by Ce addition can no longer contribute to the enhancement of wear resistance.
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