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- Ihse, Elisabet, 1977-, et al.
(författare)
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Amyloid fibrils with fragmented ATTR may be the rule in non-Val30Met ATTR amyloidosis
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Annan publikation (övrigt vetenskapligt/konstnärligt)abstract
- The clinical phenotype of familial ATTR amyloidosis depends to some extent on the particular mutation, but differences exist also within mutations. We have previously described that two types of amyloid fibril compositions exist among Swedish ATTRV30M amyloidosis patients, one consisting of a mixture of intact and fragmented ATTR (type A) and one composed of only intact ATTR (type B). Patients with type A fibrils have a late age of onset and signs of cardiomyopathy, while patients with type B fibrils have an early onset and much less myocardial involvement. The present study aimed to determine if the correlation between fibril type and clinical phenotype is true for familial amyloidosis in general. Cardiac and/or adipose tissue from 48 patients carrying 21 different non-TTRV30M mutations were examined, as well as 7 non-Swedish ATTRV30M patients. Fibril type was determined with western blotting and compared to the patients´ age of onset and degree of cardiomyopathy. Non-Swedish V30M patients showed the same correlation as described for Swedish V30M patients, with fibrils of only full-length ATTR (type B) linked to less myocardial involvement. In contrast, all patients with non-V30M mutations had a fibril composition with ATTR fragments (type A). Some of these patients had onset of disease at young age. The vast majority had increased thickness of left cardiac ventricle, but a few individuals had values within normal limits. This study shows that a fibril composition with fragmented ATTR is very common in ATTR amyloidosis. It also suggests that fibrils composed of only full-length ATTR is an exception, perhaps only found among young ATTRV30M amyloidosis patients.
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- Hernández-Prieto, Miguel Angel, 1975-, et al.
(författare)
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Imbalance in tetrapyrrole metabolites caused by deletion of scp genes in the cyanobacterium Synechocystis sp. PCC 6803 results in dramatic cellular effects
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Annan publikation (övrigt vetenskapligt/konstnärligt)abstract
- Light-harvsting like (Lil) proteins contain a chlorophyll-binding domain similar to the chlorophyll a/b binding antenna proteins of plants. The five small Cab-like proteins (SCPs) of the cyanobacterium Synechocystis sp. PCC 6803 belong to the Lil family. They have been shown to stabilize chlorophyll and to play a role in the tetrapyrrole biosynthesis pathway (Xu, H., Vavilin, D., Funk, C. and Vermaas, W. (2004) J. Biol. Chem. 279, 27971-27979). Here we show that deletion of the five scp genes in a PSI-less mutant results in a decrease of Photosystem II amount in the thylakoid membrane. This reduction of Photosystem II leads to increased cell volume, disorganized thylakoid membranes in the cell, and metabolic imbalance. We conclude that the lack of SCPs alters the assembly/repair of Photosystem II, causing an imbalance in the energetic level of the mutant cells.
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- Chumnarnsilpa, Sakesit, 1967-, et al.
(författare)
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The crystal structure of the C-terminus of adseverin: Implications for actin binding
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Annan publikation (populärvet., debatt m.m.)abstract
- Adseverin is a member of the calcium-regulated gelsolin superfamily of actin severing and capping proteins. Adseverin comprises six homologous domains (A1-A6) which share 60% homology with the six domains from gelsolin (G1-G6). Adseverin is truncated in comparison to gelsolin, lacking the C-terminal extension which masks the F-actin binding site in calcium-free gelsolin. Biochemical assays have indicated differences in the interaction of the C-terminus halves of adseverin and gelsolin with actin. Gelsolin contacts actin through a major site on G4 and a minor site on G6, while adseverin uses a site on A5. Here we present the X-ray structure of the activated C-terminal half of adseverin (A4-A6). This structure is highly similar to that of the activated form of the C-terminal half of gelsolin (G4-G6), both in arrangement of domains and in the three bound calcium ions. Comparative analysis of the actin-binding surfaces observed in the G4-G6/actin structure suggests that adseverin in this conformation will also be able to interact with actin through A4 and A6, while the A5 surface is obscured. A model of calcium-free adseverin constructed from the structure of gelsolin predicts that the interaction between A2 and A6 provides sterric inhibition to prevent interaction with F-actin in the absence of calcium. Actin-binding assays reveal that the minimal stoichiometry of adseverin to calcium needed to disassemble actin filaments is 1:1 as compared to the 1:2 that was previously observed for gelsolin. We propose that the absence of a gelsolin-like C-terminal extension in adseverin reduces the calcium requirement for activation.
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- Ericson, Y, et al.
(författare)
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Faktablad från Integrerad kustfiskövervakning: Fjällbacka (Västerhavet) 1989-2015
- 2016
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Ingår i: Faktablad 2016 från Inst för Akvatiska resurser, SLU. ; :2016:1
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Annan publikation (övrigt vetenskapligt/konstnärligt)abstract
- Faktabladet presenterar resultat och bedömningar från den integrerade kustfiskövervakningen i Fjällbacka-området 1989-2015. Övervakningen visar att situationen för tånglake i Fjällbacka är ansträngd. En återhämtning kan dock skönjas de allra sista åren. Tidigare vikande fångster och en försämring av tånglakens hälsotillstånd samt yngelstatus antyder att arten påverkas negativt av omgivningsfaktorer såsom den ökande vattentemperaturen, syrenivåer och exponering för kemikalier.
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- Ericson, Y, et al.
(författare)
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Faktablad från Integrerad kustfiskövervakning: Torhamn (södra Egentliga Östersjön) 2002-2015
- 2016
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Ingår i: Faktablad 2016 från Inst för Akvatiska resurser, SLU. ; :2016:4
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Annan publikation (övrigt vetenskapligt/konstnärligt)abstract
- Faktabladet presenterar resultat och bedömningar från den integrerade kustfiskövervakningen i Torhamnsområdet under tidsperioden 2002-2015. Resultaten från tretton års undersökningar visar en alltmer tydlig påverkan på hälsotillståndet hos abborre liknande den som observeras hos abborre och tånglake i andra kustreferensområden. Denna tydliga och successiva påverkan som ses på abborrens hälsa på individnivå har ännu inte resulterat i några påtagliga förändringar på bestånds- eller samhällsnivå.
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