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| 3. |
- Blondelle, Jordan, et al.
(författare)
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Cullin-3-dependent deregulation of ACTN1 represents a pathogenic mechanism in nemaline myopathy
- 2019
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Ingår i: JCI Insight. - : American Society for Clinical Investigation. - 2379-3708. ; 4:10
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Tidskriftsartikel (refereegranskat)abstract
- Nemaline myopathy is a congenital neuromuscular disorder characterized by muscle weakness, fiber atrophy, and presence of nemaline bodies within myofibers. However, understanding of the underlying pathomechanisms is lacking. Recently, mutations in KBTBD13, KLHL40, and KLHL41, three substrate adaptors for the E3 ubiquitin ligase Cullin-3, have been associated with early-onset nemaline myopathies. We hypothesized that deregulation of Cullin-3 and its muscle protein substrates may be responsible for disease development. Using Cullin-3-knockout mice, we identified accumulation of non-muscle α-actinins (ACTN1 and ACTN4) in muscles of these mice, which we also observed in patients with mutations in KBTBD13. Our data reveal that proper regulation of Cullin-3 activity and ACTN1 levels is essential for normal muscle and neuromuscular junction development. While ACTN1 is naturally downregulated during myogenesis, its overexpression in C2C12 myoblasts triggered defects in fusion, myogenesis, and acetylcholine receptor clustering - features that we characterized in Cullin-3-deficient mice. Taken together, our data highlight the importance of Cullin-3-mediated degradation of ACTN1 for muscle development, and indicate what is to our knowledge new pathomechanism for the etiology of myopathies seen in Cullin-3-knockout mice and patients with nemaline myopathy.
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| 5. |
- Gärling, Anita, et al.
(författare)
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SEVUP : ett datorprogram för simulering av elbilsresor
- 1997
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Ingår i: VTI:s och KFB:s forskardagar. Rapportsammanställning av föredrag vid forskardagarna 1997-01-08--09. - Linköping : Statens väg- och transportforskningsinstitut. ; , s. 37-50, s. 37-50
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Konferensbidrag (övrigt vetenskapligt/konstnärligt)
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| 7. |
- Laitila, T
(författare)
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Journalistic codes of ethics in Europe
- 1995
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Ingår i: EUROPEAN JOURNAL OF COMMUNICATION. - : SAGE PUBLICATIONS LTD. - 0267-3231. ; 10:4, s. 527-544
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)abstract
- There are over 30 national codes of ethics for journalism in force in Europe at the moment. The number of codes is greater than that of press councils - there are at present 20 functioning councils in Europe. Most of the codes have been established by the
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| 8. |
- Lewis, Christopher T. A., et al.
(författare)
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Remodeling of skeletal muscle myosin metabolic states in hibernating mammals
- 2024
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Ingår i: eLIFE. - : eLife Sciences Publications Ltd. - 2050-084X. ; 13
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Tidskriftsartikel (refereegranskat)abstract
- Hibernation is a period of metabolic suppression utilized by many small and large mammal species to survive during winter periods. As the underlying cellular and molecular mechanisms remain incompletely understood, our study aimed to determine whether skeletal muscle myosin and its metabolic efficiency undergo alterations during hibernation to optimize energy utilization. We isolated muscle fibers from small hibernators, Ictidomys tridecemlineatus and Eliomys quercinus and larger hibernators, Ursus arctos and Ursus americanus. We then conducted loaded Mant-ATP chase experiments alongside X-ray diffraction to measure resting myosin dynamics and its ATP demand. In parallel, we performed multiple proteomics analyses. Our results showed a preservation of myosin structure in U. arctos and U. americanus during hibernation, whilst in I. tridecemlineatus and E. quercinus, changes in myosin metabolic states during torpor unexpectedly led to higher levels in energy expenditure of type II, fast-twitch muscle fibers at ambient lab temperatures (20 °C). Upon repeating loaded Mant-ATP chase experiments at 8 °C (near the body temperature of torpid animals), we found that myosin ATP consumption in type II muscle fibers was reduced by 77-107% during torpor compared to active periods. Additionally, we observed Myh2 hyper-phosphorylation during torpor in I. tridecemilineatus, which was predicted to stabilize the myosin molecule. This may act as a potential molecular mechanism mitigating myosin-associated increases in skeletal muscle energy expenditure during periods of torpor in response to cold exposure. Altogether, we demonstrate that resting myosin is altered in hibernating mammals, contributing to significant changes to the ATP consumption of skeletal muscle. Additionally, we observe that it is further altered in response to cold exposure and highlight myosin as a potentially contributor to skeletal muscle non-shivering thermogenesis.
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| 9. |
- Lewis, Christopher T. A., et al.
(författare)
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Remodelling of skeletal muscle myosin metabolic states in hibernating mammals
- 2023
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Annan publikation (övrigt vetenskapligt/konstnärligt)abstract
- Hibernation is a period of metabolic suppression utilized by many small and large mammal species to survive during winter periods. As the underlying cellular and molecular mechanisms remain incompletely understood, our study aimed to determine whether skeletal muscle myosin and its metabolic efficiency undergo alterations during hibernation to optimize energy utilization. We isolated muscle fibers from small hibernators, Ictidomys tridecemlineatus and Eliomys quercinus and larger hibernators, Ursus arctos and Ursus americanus. We then conducted loaded Mant-ATP chase experiments alongside X-ray diffraction to measure resting myosin dynamics and its ATP demand. In parallel, we performed multiple proteomics analyses. Our results showed a preservation of myosin structure in U. arctos and U. americanus during hibernation, whilst in I. tridecemlineatus and E. quercinus, changes in myosin metabolic states during torpor unexpectedly led to higher levels in energy expenditure of type II, fast-twitch muscle fibers at ambient lab temperatures (20°C). Upon repeating loaded Mant-ATP chase experiments at 8°C (near the body temperature of torpid animals), we found that myosin ATP consumption in type II muscle fibers was reduced by 77-107% during torpor compared to active periods. Additionally, we observed Myh2 hyper-phosphorylation during torpor in I. tridecemilineatus, which was predicted to stabilize the myosin molecule. This may act as a potential molecular mechanism mitigating myosin-associated increases in skeletal muscle energy expenditure during periods of torpor in response to cold exposure. Altogether, we demonstrate that resting myosin is altered in hibernating mammals, contributing to significant changes to the ATP consumption of skeletal muscle. Additionally, we observe that it is further altered in response to cold exposure and highlight myosin as a potentially contributor to skeletal muscle non-shivering thermogenesis.
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- Marell, Agneta, et al.
(författare)
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Direct and indirect effects on households’ intentions to replace the old car
- 2004
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Ingår i: Journal of Retailing and Consumer Services. - 0969-6989 .- 1873-1384. ; 11:1, s. 1-8:11, s. 1-8
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Tidskriftsartikel (refereegranskat)abstract
- With the purpose of testing the hypothesis that households' intentions to replace their old car have a direct negative relationship to its perceived quality ('current level') and a direct positive relationship to their aspirations for a new car ('aspiration level'), a rotating panel of car owners were interviewed every fourth month during 2 years. In this data set the hypothesis received support. In addition the results showed that the age of the car, the total number of miles driven, and the number of anticipated repairs affected the current level, whereas marital status, the number of children, consumer confidence, and environmental concern affected the aspiration level.
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