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- Dees, M., et al.
(författare)
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Status and Future of Biomass Assessment for Energetic Use in Europe
- 2012
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Ingår i: 20th European Biomass Conference and Exhibition. - 9788889407547 ; , s. 23-24
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Konferensbidrag (refereegranskat)abstract
- Results from biomass potential assessments vary considerably, both on global and European level. On the other hand reliable figures on biomass potentials are an important basis for energy policy and for strategies that aim at an increase of use of biomass for energy both on EU-Level as well as e. g. on national level in the National Renewable Energy Action Plans (NREAPs) by the 27 member states. This paper is based on the findings of two projects, BEE and CEUBIOM. It presents an overview of the findings of these projects and includes an analysis of biomass potential aspects within the NREAPs. It presents how, based on an analysis of the status quo of potential studies, the projects developed proposals for a harmonisation of the methodologies and it presents the key areas they identified for future work in the field.
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- Smeets, C. J. P. P., et al.
(författare)
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A wireless subglacial probe for deep ice applications
- 2012
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Ingår i: Journal of Glaciology. - 0022-1430 .- 1727-5652. ; 58:211, s. 841-848
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Tidskriftsartikel (refereegranskat)abstract
- We present the design and first results from two experiments using a wireless subglacial sensor system (WiSe) that is able to transmit data through 2500 m thick ice. Energy consumption of the probes is minimized, enabling the transmission of data for at least 10 years. In July 2010 the first prototype of the system was used to measure subglacial pressure at the base and a temperature profile consisting of 23 probes in two 600 m deep holes at Russell Glacier, a land-terminating part of the West Greenland ice sheet near Kangerlussuaq. The time series of subglacial pressure show very good agreement between data from the WiSe system and the wired reference system. The wireless-measured temperature data were validated by comparison with the theoretical decrease of melting point with water pressure inside the water-filled hole directly after installation. To test the depth range of the WiSe system a second experiment using three different probe types and two different surface antennas was performed inside the 2537 m deep hole at NEEM. It is demonstrated that, with the proper combination of transmission power and surface antenna type, the WiSe system transmits data through 2500 m thick ice.
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- Wanschers, Bas F. J., et al.
(författare)
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A mutation in the human CBP4 ortholog UQCC3 impairs complex III assembly, activity and cytochrome b stability
- 2014
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Ingår i: Human Molecular Genetics. - : Oxford University Press (OUP). - 0964-6906 .- 1460-2083. ; 23:23, s. 6356-6365
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Tidskriftsartikel (refereegranskat)abstract
- Complex III (cytochrome bc(1)) is a protein complex of the mitochondrial inner membrane that transfers electrons from ubiquinol to cytochrome c. Its assembly requires the coordinated expression of mitochondrial-encoded cytochrome b and nuclear-encoded subunits and assembly factors. Complex III deficiency is a severe multisystem disorder caused by mutations in subunit genes or assembly factors. Sequence-profile-based orthology predicts C11orf83, hereafter named UQCC3, to be the ortholog of the fungal complex III assembly factor CBP4. We describe a homozygous c.59T > A missense mutation in UQCC3 from a consanguineous patient diagnosed with isolated complex III deficiency, displaying lactic acidosis, hypoglycemia, hypotonia and delayed development without dysmorphic features. Patient fibroblasts have reduced complex III activity and lower levels of the holocomplex and its subunits than controls. They have no detectable UQCC3 protein and have lower levels of cytochrome b protein. Furthermore, in patient cells, cytochrome b is absent from a high-molecular-weight complex III. UQCC3 is reduced in cells depleted for the complex III assembly factors UQCC1 and UQCC2. Conversely, absence of UQCC3 in patient cells does not affect UQCC1 and UQCC2. This suggests that UQCC3 functions in the complex III assembly pathway downstream of UQCC1 and UQCC2 and is consistent with what is known about the function of Cbp4 and of the fungal orthologs of UQCC1 and UQCC2, Cbp3 and Cbp6. We conclude that UQCC3 functions in complex III assembly and that the c.59T > A mutation has a causal role in complex III deficiency.
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