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The simulation of major midwinter stratospheric sudden warmings (SSWs) in six stratosphere-resolving general circulation models (GCMs) is examined. The GCMs are compared to a new climatology of SSWs, based on the dynamical characteristics of the events. First, the number, type, and temporal distribution of SSW events are evaluated. Most of the models show a(More)
Mutations in CHMP2B cause frontotemporal dementia (FTD) in a large Danish pedigree, which is termed FTD linked to chromosome 3 (FTD-3), and also in an unrelated familial FTD patient. CHMP2B is a component of the ESCRT-III complex, which is required for function of the multivesicular body (MVB), an endosomal structure that fuses with the lysosome to degrade(More)
[1] The El Niño–Southern Oscillation (ENSO) is the dominant mode of inter-annual variability in the tropical ocean and troposphere. Its impact on tropospheric circulation causes significant changes to the distribution of ozone. Here we derive the lower tropospheric to lower stratospheric ozone response to ENSO from observations by the Tropospheric Emission(More)
Through an international consortium, we have collected 37 tau- and TAR DNA-binding protein 43 (TDP-43)-negative frontotemporal lobar degeneration (FTLD) cases, and present here the first comprehensive analysis of these cases in terms of neuropathology, genetics, demographics and clinical data. 92% (34/37) had fused in sarcoma (FUS) protein pathology,(More)
Paroxysmal non-kinesigenic dyskinesia (PNKD) is characterized by spontaneous hyperkinetic attacks that are precipitated by alcohol, coffee, stress and fatigue. We report mutations in the myofibrillogenesis regulator 1 (MR-1) gene causing PNKD in 50 individuals from eight families. The mutations cause changes (Ala to Val) in the N-terminal region of two MR-1(More)
BACKGROUND/AIMS CHMP2B mutations are a rare cause of familial frontotemporal dementia (FTD). The clinical syndrome is dominated by personality change and behavioural symptoms, but language, memory, calculation and praxis impairments are also seen early in the course of the disease. There are no detailed studies of brain imaging in CHMP2B mutation-associated(More)
OBJECTIVE AND METHODS A longitudinal study spanning over 8 years and including 17 asymptomatic individuals with CHMP2B mutations was conducted to assess the earliest neuropsychological changes in autosomal dominant neurodegenerative disease frontotemporal dementia (FTD) linked to chromosome 3 (FTD-3). Subjects were assessed with neuropsychological tests in(More)
model climate and variability of the stratosphere. Accepted for publication in The vertical distribution of ozone instantaneous radiative forcing from satellite and chemistry climate models. Accepted for publication inmodel assessment of ozone return dates and ozone recovery in CCMVal-2 models.model assessment of the factors driving the ozone evolution over(More)
An ozone increase in the Antarctic summer stratosphere: A dynamical response to the ozone hole. 2006: Re-examination of the observed decadal variability of Earth radiation budget using altitude-corrected ERBE/ERBS non-scanner WFOV data. Climate and tropical cyclone activity: a new model downscaling approach. 2007: A globally consistent reanalysis of(More)
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