| 1. |
- Valleix, Sophie, et al.
(författare)
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D25V apolipoprotein C-III variant causes dominant hereditary systemic amyloidosis and confers cardiovascular protective lipoprotein profile
- 2016
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Ingår i: Nature Communications. - : Springer Science and Business Media LLC. - 2041-1723. ; 7
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Tidskriftsartikel (refereegranskat)abstract
- Apolipoprotein C-III deficiency provides cardiovascular protection, but apolipoprotein C-III is not known to be associated with human amyloidosis. Here we report a form of amyloidosis characterized by renal insufficiency caused by a new apolipoprotein C-III variant, D25V. Despite their uremic state, the D25V-carriers exhibit low triglyceride (TG) and apolipoprotein C-III levels, and low very-low-density lipoprotein (VLDL)/high high-density lipoprotein (HDL) profile. Amyloid fibrils comprise the D25V-variant only, showing that wild-type apolipoprotein C-III does not contribute to amyloid deposition in vivo. The mutation profoundly impacts helical structure stability of D25V-variant, which is remarkably fibrillogenic under physiological conditions in vitro producing typical amyloid fibrils in its lipid-free form. D25V apolipoprotein C-III is a new human amyloidogenic protein and the first conferring cardioprotection even in the unfavourable context of renal failure, extending the evidence for an important cardiovascular protective role of apolipoprotein C-III deficiency. Thus, fibrate therapy, which reduces hepatic APOC3 transcription, may delay amyloid deposition in affected patients.
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| 2. |
- Westlén, Daniel, 1976-
(författare)
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Why Faster is Better : On Minor Actinide Transmutation in Hard Neutron
- 2007
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Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
- In this thesis, options for efficient transmutation of transuranium elements are discussed. The focus is on plutonium, americium and curium mainly because of their long-term contribution to the radiotoxicity of spent nuclear fuel. Two innovative helium-cooled core designs are proposed, dedicated to the transmutation of actinides. The performance of the more promising of the two is studied in realistic transient fuel cycle scenarios. During the 1150 day irradiation cycle, a minor actinide consumption of 355 kg/GWth·year is achieved. An analysis of the efficiency of spallation neutron sources in helium-cooled cores is also performed. It is shown that the proton source efficiency, ψ∗, is improved by about 10% when helium is used as coolant, rather than lead-bismuth eutectic. Further, a proposal is made to transmute actinides in the upper part of a BWR core. A net consumption of transuranics is shown possible in the BWR park already when 50% of the BWR fuel is of the proposed evolutionary type. The thesis concludes that efficient transmutation of transuranic elements in dedicated helium-cooled subcritical cores is possible. But, in many instances fuel cycles without dedicated cores may lead to comparable final states. Especially, the evolutionary BWRfuel proposed seems interesting.
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