| 1. |
- Adamo, Christin S., et al.
(författare)
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EMILIN1 deficiency causes arterial tortuosity with osteopenia and connects impaired elastogenesis with defective collagen fibrillogenesis
- 2022
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Ingår i: American Journal of Human Genetics. - : Elsevier BV. - 0002-9297. ; 109:12, s. 2230-2252
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Tidskriftsartikel (refereegranskat)abstract
- EMILIN1 (elastin-microfibril-interface-located-protein-1) is a structural component of the elastic fiber network and localizes to the interface between the fibrillin microfibril scaffold and the elastin core. How EMILIN1 contributes to connective tissue integrity is not fully understood. Here, we report bi-allelic EMILIN1 loss-of-function variants causative for an entity combining cutis laxa, arterial tortuosity, aneurysm formation, and bone fragility, resembling autosomal-recessive cutis laxa type 1B, due to EFEMP2 (FBLN4) deficiency. In both humans and mice, absence of EMILIN1 impairs EFEMP2 extracellular matrix deposition and LOX activity resulting in impaired elastogenesis, reduced collagen crosslinking, and aberrant growth factor signaling. Collagen fiber ultrastructure and histopathology in EMILIN1- or EFEMP2-deficient skin and aorta corroborate these findings and murine Emilin1-/- femora show abnormal trabecular bone formation and strength. Altogether, EMILIN1 connects elastic fiber network with collagen fibril formation, relevant for both bone and vascular tissue homeostasis.
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| 2. |
- Agarwal, Pallavi, et al.
(författare)
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Collagen XII and XIV, New Partners of Cartilage Oligomeric Matrix Protein in the Skin Extracellular Matrix Suprastructure
- 2012
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Ingår i: Journal of Biological Chemistry. - 1083-351X. ; 287:27, s. 22549-22559
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Tidskriftsartikel (refereegranskat)abstract
- The tensile and scaffolding properties of skin rely on the complex extracellular matrix (ECM) that surrounds cells, vasculature, nerves, and adnexus structures and supports the epidermis. In the skin, collagen I fibrils are the major structural component of the dermal ECM, decorated by proteoglycans and by fibril-associated collagens with interrupted triple helices such as collagens XII and XIV. Here we show that the cartilage oligomeric matrix protein (COMP), an abundant component of cartilage ECM, is expressed in healthy human skin. COMP expression is detected in the dermal compartment of skin and in cultured fibroblasts, whereas epidermis and HaCaT cells are negative. In addition to binding collagen I, COMP binds to collagens XII and XIV via their C-terminal collagenous domains. All three proteins codistribute in a characteristic narrow zone in the superficial papillary dermis of healthy human skin. Ultrastructural analysis by immunogold labeling confirmed colocalization and further revealed the presence of COMP along with collagens XII and XIV in anchoring plaques. On the basis of these observations, we postulate that COMP functions as an adapter protein in human skin, similar to its function in cartilage ECM, by organizing collagen I fibrils into a suprastructure, mainly in the vicinity of anchoring plaques that stabilize the cohesion between the upper dermis and the basement membrane zone.
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| 3. |
- Agarwal, Pallavi, et al.
(författare)
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Enhanced deposition of cartilage oligomeric matrix protein is a common feature in fibrotic skin pathologies
- 2013
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Ingår i: Matrix Biology. - : Elsevier BV. - 1569-1802 .- 0945-053X. ; 32:6, s. 325-331
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Tidskriftsartikel (refereegranskat)abstract
- Skin fibrosis is characterized by activated fibroblasts and an altered architecture of the extracellular matrix. Excessive deposition of extracellular matrix proteins and altered cytokine levels in the dermal collagen matrix are common to several pathological situations such as localized scleroderma and systemic sclerosis, keloids, dermatosclerosis associated with venous ulcers and the fibroproliferative tissue surrounding invasively growing tumors. Which factors contribute to altered organization of dermal collagen matrix in skin fibrosis is not well understood. We recently demonstrated that cartilage oligomeric matrix protein (COMP) functions as organizer of the dermal collagen I network in healthy human skin (Agarwal et al., 2012). Here we show that COMP deposition is enhanced in the dermis in various fibrotic conditions. COMP levels were significantly increased in fibrotic lesions derived from patients with localized scleroderma, in wound tissue and exudates of patients with venous leg ulcers and in the fibrotic stroma of biopsies from patients with basal cell carcinoma. We postulate enhanced deposition of COMP as one of the common factors altering the supramolecular architecture of collagen matrix in fibrotic skin pathologies. Interestingly, COMP remained nearly undetectable in normally healing wounds where myofibroblasts transiently accumulate in the granulation tissue. We conclude that COMP expression is restricted to a fibroblast differentiation state not identical to myofibroblasts which is induced by TGF beta and biomechanical forces. (C) 2013 Elsevier B.V. All rights reserved.
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| 4. |
- Agvall, Björn, et al.
(författare)
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Resource use and cost implications of implementing a heart failure program for patients with systolic heart failure in Swedish primary health care
- 2014
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Ingår i: International Journal of Cardiology. - : Elsevier BV. - 0167-5273 .- 1874-1754. ; 176, s. 731-738
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Tidskriftsartikel (refereegranskat)abstract
- Aim: Heart failure (HF) is a common but serious condition which involves a significant economic burden on the health care economy. The purpose of this study was to evaluate cost and quality of life (QoL) implications of implementing a HF management program (HFMP) in primary health care (PHC).Methods and results: This was a prospective randomized open-label study including 160 patientswith a diagnosis of HF from five PHC centers in south-eastern Sweden. Patients randomized to the intervention group received information about HF from HF nurses and from a validated computer-based awareness program. HF nurses and physicians followed the patients intensely in order to optimize HF treatment according to current guidelines. The patients in the control group were followed by their regular general practitioner (GP) and received standard treatment according to local management routines. No significant changes were observed in NYHA class and quality-adjusted life years (QALY), implying that functional class and QoL were preserved. However, costs for hospital care (HC) and PHC were reduced by EUR 2167, or 33%. The total cost was EUR 4471 in the intervention group and EUR 6638 in the control group.Conclusions: Introducing HFMP in Swedish PHC in patients with HF entails a significant reduction in resource utilization and costs, and maintains QoL. Based on these results, a broader implementation of HFMP in PHC may be recommended. However, results should be confirmed with extended follow-up to verify long-term effects.
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| 5. |
- Bourelius, Lars, et al.
(författare)
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Öppna digitala resurser (OER)
- 2012
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Ingår i: Nyheter & Debatt. - 1651-3363. ; :3, s. 4-4
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)abstract
- Lärande sker i sociala sammanhang. Genom nätet är klassrummet idag globalt. Tusentals universitet, skolor och lärare i världen delar idag med sig av kursmaterial, föreläsningar, experiment, reportage och bilder så att andra kan återanvända materialet i sin undervisning.
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| 6. |
- Danielson, Mats, et al.
(författare)
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Utilizing Surrogate Numbers for Probability Elicitation
- 2018
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Ingår i: Decision Making. - : IntechOpen. - 9781789237955 - 9781789237962 ; , s. 39-56
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Bokkapitel (övrigt vetenskapligt/konstnärligt)abstract
- Comparatively few of the vast number of suggested decision-analytical methods have been widely spread in actual practice. The majority of those methods call for exact and accurate numbers as input, which could be one of several reasons for this lack of actual use; people frequently seem to be unfamiliar with, or reluctant to express those, in a sense, “true” values required. Many alternative methods to resolve this complication have been suggested over the years, including procedures for dealing with incomplete information. One way, which has proliferated for a while, is to introduce so-called surrogate numbers in the form of ordinal ranking methods for multi-criteria weights. In this chapter, we show how those can be adapted for use in probability elicitation. Furthermore, when decision-makers possess more information regarding the relative strengths of probabilities, that is, some form of cardinality, the input information to ordinal methods is sometimes too restricted. Therefore, we suggest a testing methodology and analyze the relevance of a set of cardinal ordering methods in addition to the ordinal ones.
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| 7. |
- Enberg, Sofia, et al.
(författare)
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Mapping and modelling of optical properties from pulp to super calendered paper
- 2015
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Ingår i: Appita journal. - 1038-6807. ; 68:2, s. 128-138-
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Forskningsöversikt (refereegranskat)abstract
- During production of mechanical pulp based paper, the pulp darkenswithin the process which represents a significant cost for the mill. Amapping of the optical properties of the pulp was conducted along theprocess, from pulp mill to paper machine, at a mill producing supercalendered (SC) paper on three paper machines. The focus was to evaluateat which positions in the mill the discolouration occurs and also whichfraction of the pulp darkens the most.The fine fraction of the pulp darkened more than the long fibrefraction. Bleached pulp diluted with white water from the paper machinedarkened more during storage than pulp diluted with clear filtrate. Thewhite water contains a considerable amount of suspended solids, i.e.pulp fines and clay. There was a tendency for a shoulder in absorptionspectra of the pulp stored in white water from the paper machine in theregion 550 to 650 nm, both for fibres and for fines. This is in the sameregion where the added dyes have their absorption maxima and also somecomplexes between iron and some of the components among lignin and theextractives. Simulation of retention times of different fractions showedthat, although the main part of the fine material is retained in thepaper within a few hours, a small part might circulate for considerablylonger time.
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| 8. |
- Enberg, Sofia, et al.
(författare)
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Some causes of formation of colour during storage of hydrogen-peroxide bleached Norway spruce mechanical pulp
- 2014
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Ingår i: Nordic Pulp & Paper Research Journal. - 0283-2631 .- 2000-0669. ; 29:2, s. 356-366
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Tidskriftsartikel (refereegranskat)abstract
- The discolouration of hydrogen-peroxide bleached Norway spruce mechanical pulp during storage in mill systems was studied and the contributions of process water, iron and dyes were evaluated over the visible spectrum. Washing of the pulp made it less sensitive to storage, possibly due to the removal of extractives, lignin-like substances, iron and pulp fines. Storage in white water gave extensive discolouration with a shoulder in the absorption spectrum at around 550-650 nm. Most of the colour was associated with pulp fines or filler but some colour was also found in smaller fractions and in the water phase. The addition of ferric ions increased the light absorption coefficient during storage, initially at short wavelengths and then over the whole spectrum, but could not explain the increased absorption at 550-650 nm and could not be the only cause of the darkening in the mill system. A cationic basic violet dye gave a shoulder in the absorption spectrum similar to that in the mill system, but the absorption in this area did not increase during storage. Model calculations indicate that ferric ions together with violet and red dyes could explain a major part, but not all, of the colour observed in the mill system after storage. The darkening not accounted for at longer wavelengths and around 550-650 nm is suggested to be related to fines and fillers including dissolved and colloidal substances associated with these particles.
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| 9. |
- Enberg, Sofia
(författare)
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Storage of Hydrogen Peroxide Bleached Mechanical Pulp : Reduction in Reflectance over the Visible Spectrum
- 2014
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Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
- The objective of this thesis is to determine possible causes of the darkening of hydrogen peroxide bleached mechanical pulp over the visible spectrum and their relative contributions. It focuses on both process conditions and the composition of the pulp and the dilution water, including additions or losses of material along the process line from the bleach tower to the paper machine.A mapping of the optical properties of the pulp along the process showed that the fine fraction of the pulp darkened more than the long fibre fraction. Simulation of retention times of different fractions showed that the main part of the fine material is retained in the paper within a few hours, a small part might circulate for considerably longer time and may therefore be strongly coloured.Storage trials were mainly performed using a hydrogen peroxide bleached mechanical pulp intended for SC paper made of Norway spruce (Picea abies), sampled on one occasion and stored in a freezer. Unwashed or well-washed pulp was stored in distilled water or in different process waters. Some complementary trials were included, e.g. unbleached pulp.Time and temperature were the process variables that gave the strongest darkening of the pulp, as expected, both in a clean and a more process-like system, whereas pH only had an effect in the presence of process waters; the highest brightness stability was seen at a pH around 5.5–6.0.The darkening was due to an increase in the light absorption coefficient (k) beginning at short wavelengths, but after longer storage times the increase in kλ also became noticeable at longer wavelengths. The colour (CIE L*, a*, b*) of the pulp changed towards red and yellow, initially more towards red and then more towards yellow. These changes were clearly visible.Washing of the bleached pulp made it less sensitive to storage; possibly due to the removal of extractives, lignin-like substances, metals and pulp fines. This washing had little effect before storage and the amount of material removed was small.The pulp darkened more when stored in process waters compared to distilled water. Apart from fibres, most of the colour was associated with pulp fines or filler but some colour was also found in the dissolved and colloidal fractions. At an increased pulp consistency, the increase in k460 was smaller.Storage in white water from the paper machine gave extensive discolouration with a shoulder in the absorption spectrum around 550–650 nm, which increased with time. The addition of ferric ions increased the light absorption coefficient during storage, but could not explain the increased absorption at 550–650 nm nor could it be the only cause of the darkening in the mill system. A cationic basic violet dye gave a shoulder in the absorption spectrum similar to that of the mill system, but the absorption of the dye did not increase during storage. Model calculations indicate, but do not prove, that ferric ions together with violet and red dyes could have played a major, but not exclusive role in the colour observed in the mill system after storage. The darkening not accounted for, at longer wavelengths and around 550–650 nm, is suggested to be related to fines and fillers including dissolved and colloidal substances associated with these particles.A method to produce representative sheets for determination of optical properties of mechanical pulps was developed. The new method makes it possible to follow changes in light absorption and light scattering coefficients over the visible range of wavelengths. It is approximately six times faster than standard methods, reduces the risk of additional darkening of the sample and can be used with small pulp quantities.The deviation from the expected linear behaviour of the light scattering coefficient, s, at wavelengths corresponding to strong light absorption has been studied using the Kubelka-Munk model and the angular resolved DORT2002 radiative transfer solution method. The decrease in s could not be explained by errors introduced in the Kubelka-Munk modelling by anisotropic scattering.
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| 10. |
- Enberg, Sofia, et al.
(författare)
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The influence of process conditions during pulp storage on the optical properties of Norway spruce high-yield pulps
- 2009
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Ingår i: Proceedings - 2009 International Mechanical Pulping Conference, IMPC 2009. ; , s. 117-121
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Konferensbidrag (refereegranskat)abstract
- The present study is part of a larger work aiming at establishing the knowledge basis needed to develop cost-effective processes for producing high-bright mechanical and chemimechanical pulps. The influence of process conditions (time, pH, temperature, consistency) on the optical properties during pulp storage of a well-washed hydrogen peroxide bleached Norway spruce pulp has been examined. The general effect of storage was darkening of the pulp due to an increase in light absorption, especially at shorter wavelengths. Time and temperature were found to be the process variables that had the largest impact, the trends were similar independent of pH and pulp consistency. The light scattering ability increased initially, giving a positive contribution to brightness. This indicates that the storage also resulted in a change in sheet structure.
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