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- Low, W C, et al.
(author)
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Hereditary multi-infarct dementia of the Swedish type is a novel disorder different from NOTCH3 causing CADASIL
- 2007
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In: Brain. - : Oxford University Press (OUP). - 0006-8950 .- 1460-2156. ; 130:Part 2, s. 357-367
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Journal article (peer-reviewed)abstract
- Several hereditary small vessel diseases (SVDs) of the brain have been reported in recent years. In 1977, Sourander and Wålinder described hereditary multi-infarct dementia (MID) in a Swedish family. In the same year, Stevens and colleagues reported chronic familial vascular encephalopathy in an English family bearing a similar phenotype. These disorders have invariably been suggested to be cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) but their genetic identities remain unknown. We used molecular, radiological and neuropathological methods to characterize these disorders. Direct DNA sequencing unexpectedly confirmed that affected members of the English family carried the R141C mutation in the NOTCH3 gene diagnostic of CADASIL. However, we did not detect any pathogenic mutations in the entire 8091 bp reading frame of NOTCH3 or find clear evidence for NOTCH3 gene linkage in the Swedish DNA. This was consistent with the lack of hyperintense signals in the anterior temporal pole and external capsule in Swedish subjects upon magnetic resonance imaging. We further found no evidence for granular osmiophilic material in skin biopsy or post-mortem brain samples of affected members in the Swedish family. In addition, there was distinct lack of NOTCH3 N-terminal fragments in the cerebral microvasculature of the Swedish hereditary MID subjects compared to the intense accumulation in the English family afflicted with CADASIL. Several differences in arteriosclerotic changes in both the grey and white matter were also noted between the disorders. The sclerotic index values, density of collagen IV immunoreactivity in the microvasculature and number of perivascular macrophages were greater in the English CADASIL samples compared to those from the Swedish brains. Multiple approaches suggest that the Swedish family with hereditary MID suspected to be CADASIL has a different novel disorder with dissimilar pathological features and belongs to the growing number of genetically uncharacterized familial SVDs.
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- Stirling, R., et al.
(author)
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Global survey on durability variation – on the effect of the reference species
- 2016
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In: Proceedings of the International Research Group Annual Meeting 2016.
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Conference paper (other academic/artistic)abstract
- Climate change due to anthropogenic emissions is the largest environmental challenge of ourtime. Forest-based value chains play an important role in reducing the accumulation of CO2 in the atmosphere. Maximizing the use of wood to tackle climate change requires improved understanding of the service life of timber products. This information can best be obtained from field testing and while there is an abundance of field performance data from sites all over the world, most of the data are not available in a form that can be utilised for service life models.The IRG Durability Database aims to improve the usability of existing performance data and create added value for durability research and service life prediction. The present paper takes the first steps in comparing global field test performance data from the IRG Durability Database for non-durable reference species. Data were obtained from six species above ground and ground contact field tests from 36 sites around the world. For each dataset, decay rates and service life (where applicable) were calculated. Datasets were then grouped together based on test method and species. Decay rate was faster and more uniform in ground contact than above ground. Inground contact, beech decayed most rapidly, followed by Norway spruce and Scots pines apwood. All appeared to be suitable for use as reference species, however slow-grown spruce should be avoided. There were no statistically significant correlations between ground contact decay rate and the Scheffer Climate Index (SCI). In above ground tests, differences in decay ratewere largely related to differences in moisture dynamics. Species with the greatest absorption and retention of water decayed most rapidly. Test methods that absorbed and retained the most moisture (e.g. painted L-joints) resulted in more rapid decay. Above ground decay rate and SCI were significantly correlated in two data sets that had a wide range of SCI values. Correlations were not significant when only European test sites were included. Estimating decay rate from field testing results in highly variable data. Comparing data from global test sites is made more difficult by the absence of common field testing standards.
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