996 resultados para Malmberg, Jakob Johan


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Summary: The abandonment of foreign graphemes in Early Modern Finnish with special reference to Jakob Johan Malmberg's works

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Invocatio: [hepreaa].

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Johan Jakob Nervander f. 23.2.1805 i Nystad d. 15.3.1848 i Helsingfors Johan Jakob Nervander var finländsk diktare, fysiker och meteorolog, samt en nyckelfigur i Lördagssällskapet. Hans inflytande inom den finländska kultureliten var som störst på 1820-40-talen. Fast den vetenskapliga forskningen krävde största delen av Nervanders uppmärksamhet, drogs han också till diktandet. Nervanders viktigaste verk som poet är Jephtas Bok, En Minnes-Sång i Israel, som belönades med Svenska Akademiens andra pris år 1832 och trycktes åtta år senare. Nervanders lyrik var formmässigt slipad. Till innehåll och stämning varierade dikterna kraftig, från starka känslouttryck och fritt flygande fantasi till melankoli och inbundenhet. http://www.blf.fi/artikel.php?id=3567 http://www.kansallisbiografia.fi/kb/artikkeli/3567/

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BACKGROUND: Prion diseases are a group of invariably fatal neurodegenerative disorders affecting humans and a wide range of mammals. An essential part of the infectious agent, termed the prion, is composed of an abnormal isoform (PrPSc) of a host-encoded normal cellular protein (PrPC). The conversion of PrPC to PrPSc is thought to play a crucial role in the development of prion diseases and leads to PrPSc deposition, mainly in the central nervous system. Sporadic Creutzfeldt-Jakob disease (sCJD), the most common form of human prion disease, presents with a marked clinical heterogeneity. This diversity is accompanied by a molecular signature which can be defined by histological, biochemical, and genetic means. The molecular classification of sCJD is an important tool to aid in the understanding of underlying disease mechanisms and the development of therapy protocols. Comparability of classifications is hampered by disparity of applied methods and inter-observer variability. METHODS AND FINDINGS: To overcome these difficulties, we developed a new quantification protocol for PrPSc by using internal standards on each Western blot, which allows for generation and direct comparison of individual PrPSc profiles. By studying PrPSc profiles and PrPSc type expression within nine defined central nervous system areas of 50 patients with sCJD, we were able to show distinct PrPSc distribution patterns in diverse subtypes of sCJD. Furthermore, we were able to demonstrate the co-existence of more than one PrPSc type in individuals with sCJD in about 20% of all patients and in more than 50% of patients heterozygous for a polymorphism on codon 129 of the gene encoding the prion protein (PRNP). CONCLUSION: PrPSc profiling represents a valuable tool for the molecular classification of human prion diseases and has important implications for their diagnosis by brain biopsy. Our results show that the co-existence of more than one PrPSc type might be influenced by genetic and brain region-specific determinants. These findings provide valuable insights into the generation of distinct PrPSc types.

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Kirje

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Kirje 23.1.1968

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Alzheimer"s disease and prion pathologies (e.g., Creutzfeldt-Jakob disease) display profound neural lesions associated with aberrant protein processing and extracellular amyloid deposits. For APP processing, emerging data suggest that the adaptor protein Dab1 plays a relevant role in regulating its intracellular trafficking and secretase-mediated proteolysis. Although some data have been presented, a putative relationship between human prion diseases and Dab1/APP interactions is lacking. Therefore, we have studied the putative relation between Dab1, APP processing and Aβ deposition, targets in sCJD cases. Our biochemical results categorized two groups of sCJD cases, which also correlated with PrPsc types 1 and 2 respectively. One group, with PrPsc type 1 showed increased Dab1 phosphorylation, and lower βCTF production with an absence of Aβ deposition. The second sCJD group, which carried PrPsc type 2, showed lower levels of Dab1 phosphorylation and βCTF production, similar to control cases. Relevant Aβ deposition in the second sCJD group was measured. Thus, a direct correlation between Dab1 phosphorylation, Aβ deposition and PrPsc type in human sCJD is presented for the first time.

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Donateur : Reclus, Élisée (1830-1905)