| 1. |
- Donkervoort, S., et al.
(författare)
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Pathogenic Variants in the Myosin Chaperone UNC-45B Cause Progressive Myopathy with Eccentric Cores
- 2020
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Ingår i: American Journal of Human Genetics. - : Elsevier BV. - 0002-9297. ; 107:6, s. 1078-1095
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Tidskriftsartikel (refereegranskat)abstract
- The myosin-directed chaperone UNC-45B is essential for sarcomeric organization and muscle function from Caenorhabditis elegans to humans. The pathological impact of UNC-45B in muscle disease remained elusive. We report ten individuals with bi-allelic variants in UNC45B who exhibit childhood-onset progressive muscle weakness. We identified a common UNC45B variant that acts as a complex hypomorph splice variant. Purified UNC-45B mutants showed changes in folding and solubility. In situ localization studies further demonstrated reduced expression of mutant UNC-45B in muscle combined with abnormal localization away from the A-band towards the Z-disk of the sarcomere. The physiological relevance of these observations was investigated in C. elegans by transgenic expression of conserved UNC-45 missense variants, which showed impaired myosin binding for one and defective muscle function for three. Together, our results demonstrate that UNC-45B impairment manifests as a chaperonopathy with progressive muscle pathology, which discovers the previously unknown conserved role of UNC-45B in myofibrillar organization.
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| 2. |
- Ghadimi, P., et al.
(författare)
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Simulation of flow over a confined square cylinder and optimal passive control of vortex shedding using a detached splitter plate
- 2015
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Ingår i: Scientia Iranica. International Journal of Science and Technology. - : Sharif University of Technology. - 1026-3098 .- 2345-3605. ; 22:1, s. 175-186
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Tidskriftsartikel (refereegranskat)abstract
- In the area of bluff body aerodynamics, controlling the vortex shedding is of great importance to prevent damages caused by the vortex-induced forces. Accordingly, a short and thin splitter plate has been utilized at different downstream positions to interfere with the vortex wake of a confined square cylinder in the framework of an optimal passive control study. Vorticity-stream function formulation and finite-difference method have been used to numerically simulate the two-dimensional laminar flow. For preventing the flow from becoming three-dimensional, the Reynolds number is kept below 250. Validation of the code and mesh dependency analysis are offered. Based on the flow patterns acquired, the optimal position of the splitter plate behind the cylinder can prevent the detachment of the pair of vortices from the cylinder and will keep the streamlines symmetric around the zero streamline. Consequently, vortex shedding does not occur and the vortex-induced forces are eliminated entirely. Interestingly we showed that the vortex can be suppressed even when the splitter plate is asymmetrically arranged behind the cylinder, especially in the case of positioning the body close to the surrounding walls.
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| 3. |
- Ghadimi, P., et al.
(författare)
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Study of water entry of circular cylinder by using analytical and numerical solutions
- 2012
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Ingår i: Journal of the Brazilian Society of Mechanical Sciences and Engineering. - : Springer Nature. - 1678-5878 .- 1806-3691. ; 34:3, s. 225-232
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Tidskriftsartikel (refereegranskat)abstract
- Water impact phenomenon in the case of a circular cylinder is an important issue in offshore industry where cross members may be in the splash zone of the incident wave. An analytical method as well as a numerical solution are employed to study the water entry problem of a circular section. The procedure for derivation of the analytical formulas is demonstrated step by step. The volume of fluid (VOF) simulation of the water entry problem is also performed to offer comparison of the results of the linearized analytical solution with a fully nonlinear and viscous fluid flow solution. To achieve this, the FLOW- 3D code is utilized. Some consideration has also been given to the points of intersection of the free surface and the body, where the singularities exist in the free surface deformation and velocities, as predicted by the linear theory. These singularities appear to be avoided in the real fluid by the formation of jets which quickly break up into sprays under the action of surface tension. Slamming force, free surface profile, impact force, pressure distribution and evolution of intersection points are also presented and comparisons of the obtained results against the results of previous studies illustrate favorable agreements.
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