47 research outputs found

    Structural and biophysical studies of adhesive binding by classical cadherins

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    Fiscal policy, macroeconomic performance and industry structure in a small open economy

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    We analyse how fiscal policy affects both the macroeconomy and the industry structure, using a multi-sector macroeconomic model of the Norwegian economy with an inflation targeting monetary policy. Our simulations show that the government spending multiplier in the case of a permanent expansionary fiscal policy coupled with a Taylor-type interest rate rule is around 1 over a ten-year horizon. The corresponding labour tax multiplier is about 0.5. These multipliers are somewhat larger in the case of a transitory fiscal stimulus. The government spending multiplier, in the case of either a permanent or a transitory fiscal stimulus, is considerably larger than 1 when monetary policy is made accommodative by keeping the interest rate fixed. Our simulations also show that the industry structure is substantially affected by an expansionary fiscal policy, as value added in the non-traded goods sector increases at the expense of value added in the traded goods sector. The contraction of activity in the traded goods sector increases when monetary tightening accompanies the fiscal stimulus. Hence, we find that such a policy mix is likely to produce significant de-industrialisation in a small open economy with inflation targeting

    Fiscal policy, macroeconomic performance and industry structure in a small open economy

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    We analyse how fiscal policy affects both the macroeconomy and the industry structure, using a multi-sector macroeconomic model of the Norwegian economy with an inflation targeting monetary policy. Our simulations show that the magnitude of the government spending and labour tax cut multipliers, whether monetary policy is active or passive, is comparable to what is found in the literature. A novel finding from our simulations is that the industry structure is substantially affected by an expansionary fiscal policy, as value added in the non-traded goods sector increases at the expense of value added in the traded goods sector. Moreover, expansionary fiscal policy reduces the mark-ups in the traded goods sector, while the mark-ups are roughly unchanged in the non-traded goods sector. The contraction of activity in the traded goods sector increases when monetary tightening accompanies the fiscal stimulus. Hence, we find that such a policy mix is likely to produce significant de-industrialization in a small open economy with inflation targeting.publishedVersio

    COVID-19, tapt verdiskaping og finanspolitikkens rolle. Utredning for Koronakommisjonen

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    Ifølge våre beregninger førte pandemien til at BNP for Fastlands-Norge i 2020 falt med 145 milliarder kroner, eller 4,7 prosent, sammenlignet med en bane uten korona. Samlet for perioden 2020–2023 anslås det neddiskonterte tapet til rundt 330 milliarder kroner, tilsvarende 11 prosent av årlig BNP for Fastlands-Norge. Anslaget er svært usikkert. Ettersom pandemien fullstendig har dominert verdensøkonomien siden midten av mars, har vi med få unntak lagt til grunn at avvik fra prognosen som ble laget i desember 2019 skyldes korona

    Hermansky-Pudlak syndrome type 2 manifests with fibrosing lung disease early in childhood

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    Background: Hermansky-Pudlak syndrome (HPS), a hereditary multisystem disorder with oculocutaneous albinism, may be caused by mutations in one of at least 10 separate genes. The HPS-2 subtype is distinguished by the presence of neutropenia and knowledge of its pulmonary phenotype in children is scarce. Methods: Six children with genetically proven HPS-2 presented to the chILD-EU register between 2009 and 2017; the data were collected systematically and imaging studies were scored blinded. Results: Pulmonary symptoms including dyspnea, coughing, need for oxygen, and clubbing started 3.3 years before the diagnosis was made at the mean age of 8.83 years (range 2-15). All children had recurrent pulmonary infections, 3 had a spontaneous pneumothorax, and 4 developed scoliosis. The frequency of pulmonary complaints increased over time. The leading radiographic pattern was ground-glass opacities with a rapid increase in reticular pattern and traction bronchiectasis between initial and follow-up Computer tomography (CT) in all subjects. Honeycombing and cysts were newly detectable in 3 patients. Half of the patients received a lung biopsy for diagnosis; histological patterns were cellular non-specific interstitial pneumonia, usual interstitial pneumonia-like, and desquamative interstitial pneumonia. Conclusions: HPS-2 is characterized by a rapidly fibrosing lung disease during early childhood. Effective treatments are required

    Hermansky-Pudlak syndrome type 2 manifests with fibrosing lung disease early in childhood

    Get PDF
    Background: Hermansky-Pudlak syndrome (HPS), a hereditary multisystem disorder with oculocutaneous albinism, may be caused by mutations in one of at least 10 separate genes. The HPS-2 subtype is distinguished by the presence of neutropenia and knowledge of its pulmonary phenotype in children is scarce. Methods: Six children with genetically proven HPS-2 presented to the chILD-EU register between 2009 and 2017;the data were collected systematically and imaging studies were scored blinded. Results: Pulmonary symptoms including dyspnea, coughing, need for oxygen, and clubbing started 3.3 years before the diagnosis was made at the mean age of 8.83 years (range 2-15). All children had recurrent pulmonary infections, 3 had a spontaneous pneumothorax, and 4 developed scoliosis. The frequency of pulmonary complaints increased over time. The leading radiographic pattern was ground-glass opacities with a rapid increase in reticular pattern and traction bronchiectasis between initial and follow-up Computer tomography (CT) in all subjects. Honeycombing and cysts were newly detectable in 3 patients. Half of the patients received a lung biopsy for diagnosis;histological patterns were cellular non-specific interstitial pneumonia, usual interstitial pneumonia-like, and desquamative interstitial pneumonia. Conclusions: HPS-2 is characterized by a rapidly fibrosing lung disease during early childhood. Effective treatments are required

    CUX1-related neurodevelopmental disorder: deep insights into phenotype-genotype spectrum and underlying pathology

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    Heterozygous, pathogenic CUX1 variants are associated with global developmental delay or intellectual disability. This study delineates the clinical presentation in an extended cohort and investigates the molecular mechanism underlying the disorder in a Cux1+/− mouse model. Through international collaboration, we assembled the phenotypic and molecular information for 34 individuals (23 unpublished individuals). We analyze brain CUX1 expression and susceptibility to epilepsy in Cux1+/− mice. We describe 34 individuals, from which 30 were unrelated, with 26 different null and four missense variants. The leading symptoms were mild to moderate delayed speech and motor development and borderline to moderate intellectual disability. Additional symptoms were muscular hypotonia, seizures, joint laxity, and abnormalities of the forehead. In Cux1+/− mice, we found delayed growth, histologically normal brains, and increased susceptibility to seizures. In Cux1+/− brains, the expression of Cux1 transcripts was half of WT animals. Expression of CUX1 proteins was reduced, although in early postnatal animals significantly more than in adults. In summary, disease-causing CUX1 variants result in a non-syndromic phenotype of developmental delay and intellectual disability. In some individuals, this phenotype ameliorates with age, resulting in a clinical catch-up and normal IQ in adulthood. The post-transcriptional balance of CUX1 expression in the heterozygous brain at late developmental stages appears important for this favorable clinical course.CAG was supported by the Eunice Kennedy Shriver National Institute Of Child Health & Human Development of the National Institutes of Health under Award Number P50 HD103525. This work was funded by PID2020-112831GB-I00 AEI /10.13039/501100011033 (MN). SS was supported by a grant from the NIH/NINDS (K23NS119666). SWS is supported by the Hospital for Sick Children Foundation, Autism Speaks, and the University of Toronto McLaughlin Center. EM-G was supported by a grant from MICIU FPU18/06240. EVS. was supported by a grant from the NIH (EY025718). CRF was supported by the fund to support clinical research careers in the Region of Southern Denmark (Region Syddanmarks pulje for kliniske forskerkarriereforløb).Peer reviewe

    Floodable Urban Landscapes for a Resilient City: Potential for the City of Seattle

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    Thesis (Master's)--University of Washington, 2019As a professional project for Seattle Public Utilities, this thesis explores the potential of the urban landscape to act as an integral component of the water infrastructure system as a climate adaptation strategy that simultaneously creates dynamic, multifunctional public open spaces. As an investigation in support of SPU’s Integrated System Plan, this thesis explores the following questions: • What can examples of floodable spaces beyond the context of Seattle teach us about how effectively they deliver functional drainage performance along with usable open space? • How could Seattle benefit from this concept? • What might be priority locations for floodable spaces in the City of Seattle? • How might a concept for floodable spaces in the Aurora-Licton urban village demonstrate how recreational space can work in tandem with drainage function for climate resilience, ecological health, and quality of life improvements
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