The ionized and hot gas in M17 SW: SOFIA/GREAT THz observations of [C II] and 12CO J=13-12

With new THz maps that cover an area of ~3.3x2.1 pc^2 we probe the spatial distribution and association of the ionized, neutral and molecular gas components in the M17 SW nebula. We used the dual band receiver GREAT on board the SOFIA airborne telescope to obtain a 5'.7x3'.7 map of the 12CO J=13-12 transition and the [C II] 158 um fine-structure line in M17 SW and compare the spectroscopically resolved maps with corresponding ground-based data for low- and mid-J CO and [C I] emission. For the first time SOFIA/GREAT allow us to compare velocity-resolved [C II] emission maps with molecular tracers. We see a large part of the [C II] emission, both spatially and in velocity, that is completely non-associated with the other tracers of photon-dominated regions (PDR). Only particular narrow channel maps of the velocity-resolved [C II] spectra show a correlation between the different gas components, which is not seen at all in the integrated intensity maps. These show different morphology in all lines but give hardly any information on the origin of the emission. The [C II] 158 um emission extends for more than 2 pc into the M17 SW molecular cloud and its line profile covers a broader velocity range than the 12CO J=13-12 and [C I] emissions, which we interpret as several clumps and layers of ionized carbon gas within the telescope beam. The high-J CO emission emerges from a dense region between the ionized and neutral carbon emissions, indicating the presence of high-density clumps that allow the fast formation of hot CO in the irradiated complex structure of M17 SW. The [C II] observations in the southern PDR cannot be explained with stratified nor clumpy PDR models.Comment: 4 pages, 4 figures, letter accepted for the SOFIA/GREAT A&A 2012 special issu

Bi-allelic JAM2 Variants Lead to Early-Onset Recessive Primary Familial Brain Calcification.

Primary familial brain calcification (PFBC) is a rare neurodegenerative disorder characterized by a combination of neurological, psychiatric, and cognitive decline associated with calcium deposition on brain imaging. To date, mutations in five genes have been linked to PFBC. However, more than 50% of individuals affected by PFBC have no molecular diagnosis. We report four unrelated families presenting with initial learning difficulties and seizures and later psychiatric symptoms, cerebellar ataxia, extrapyramidal signs, and extensive calcifications on brain imaging. Through a combination of homozygosity mapping and exome sequencing, we mapped this phenotype to chromosome 21q21.3 and identified bi-allelic variants in JAM2. JAM2 encodes for the junctional-adhesion-molecule-2, a key tight-junction protein in blood-brain-barrier permeability. We show that JAM2 variants lead to reduction of JAM2 mRNA expression and absence of JAM2 protein in patient's fibroblasts, consistent with a loss-of-function mechanism. We show that the human phenotype is replicated in the jam2 complete knockout mouse (jam2 KO). Furthermore, neuropathology of jam2 KO mouse showed prominent vacuolation in the cerebral cortex, thalamus, and cerebellum and particularly widespread vacuolation in the midbrain with reactive astrogliosis and neuronal density reduction. The regions of the human brain affected on neuroimaging are similar to the affected brain areas in the myorg PFBC null mouse. Along with JAM3 and OCLN, JAM2 is the third tight-junction gene in which bi-allelic variants are associated with brain calcification, suggesting that defective cell-to-cell adhesion and dysfunction of the movement of solutes through the paracellular spaces in the neurovascular unit is a key mechanism in CNS calcification

Accumulating discursive capital, valuating subject positions. From Marx to Foucault

Whenever people use language, they participate in valuation practices, i.e. they give value to themselves as well as to others. To account for the construction of social inequality through discursive valuation practices, discourse theorists need Marxist theory and Marxists need discourse theory. By going from the early Marx to the late Foucault, I will revisit Marx?s value theory in light of practice-oriented approaches to social inequality. I will discuss examples from two distinct arenas, the monopolization of attention by populist leaders and the academic star system, both of which are accounted for in terms of the accumulation of discursive capital. This perspective asks how the value of subject positions is constructed and hierarchies between them are established in discursive practices. Investigating the construction of valuable subject positions in discourse communities, this perspective attempts to overcome the traditional division between language, the economic and the social. Discourse not only represents value and the social order but, through representation, it also contributes to constituting the social as a hierarchical world of more or less valued subject positions

Early-infantile onset epilepsy and developmental delay caused by bi-allelic GAD1 variants

Gamma-aminobutyric acid (GABA) and glutamate are the most abundant amino acid neurotransmitters in the brain. GABA, an inhibitory neurotransmitter, is synthesized by glutamic acid decarboxylase (GAD). Its predominant isoform GAD67, contributes up to ∼90% of base-level GABA in the CNS, and is encoded by the GAD1 gene. Disruption of GAD1 results in an imbalance of inhibitory and excitatory neurotransmitters, and as Gad1−/− mice die neonatally of severe cleft palate, it has not been possible to determine any potential neurological dysfunction. Furthermore, little is known about the consequence of GAD1 disruption in humans. Here we present six affected individuals from six unrelated families, carrying bi-allelic GAD1 variants, presenting with developmental and epileptic encephalopathy, characterized by early-infantile onset epilepsy and hypotonia with additional variable non-CNS manifestations such as skeletal abnormalities, dysmorphic features and cleft palate. Our findings highlight an important role for GAD1 in seizure induction, neuronal and extraneuronal development, and introduce GAD1 as a new gene associated with developmental and epileptic encephalopathy

Parent perceptions of the quality of life of pet dogs living with neuro-typically developing and neuro-atypically developing children: an exploratory study

There is growing scientific and societal recognition of the role that pet dogs can play in healthy development of children; both those who are neuro-typically developing and those who live with a neuro-developmental disorder, such as autism or attention deficit hyperactivity disorder. However, little attention has been paid to how living with children positively and negatively affects quality of life of a pet dog. In this exploratory study we conducted semi-structured interviews with parents of neuro-typically developing children (n = 18) and those with a neuro-developmental disorder (n = 18) who owned a pet dog, until no new factors were identified. Living with children brought potentially positive benefits to the dog’s life including: imposition of a routine, participation in recreational activities and the development of a strong bond between the child and the dog. The importance of maintaining a routine was particularly prevalent in families with children with neuro-developmental disorders. Potential negative factors included having to cope with child meltdowns and tantrums, over stimulation from child visitors, harsh contact and rough and tumble play with the child. The regularity and intensity of meltdowns and tantrums was particularly evident in responses from parents with children with a neuro-developmental disorder. However, child visitors and rough play and contact were mentioned similarly across the groups. Protective factors included having a safe haven for the dog to escape to, parent’s awareness of stress signs and child education in dog-interaction. Parents were also asked to complete a stress response scale to provide an initial quantitative comparison of stress responses between dogs living with the two family-types. Parents with neuro-typically developing children more frequently observed their dog rapidly running away from a situation and less frequently observed their dog widening their eyes, than parents with children with a neuro-developmental disorder. We propose the development of a stress audit based on the findings reported here, to prevent potential dangerous situations, which may lead to dog bites and dog relinquishment and allow owners to maximise the benefits of dog ownership

Performance of the CMS Cathode Strip Chambers with Cosmic Rays

The Cathode Strip Chambers (CSCs) constitute the primary muon tracking device in the CMS endcaps. Their performance has been evaluated using data taken during a cosmic ray run in fall 2008. Measured noise levels are low, with the number of noisy channels well below 1%. Coordinate resolution was measured for all types of chambers, and fall in the range 47 microns to 243 microns. The efficiencies for local charged track triggers, for hit and for segments reconstruction were measured, and are above 99%. The timing resolution per layer is approximately 5 ns

Politik mit dem Einkaufswagen : Unternehmen und Konsumenten als Bürger in der globalen Mediengesellschaft

Forschungsprojekt gefördert durch die DFGEine gegenseitige Durchdringung von Zivilgesellschaft und Markt manifestiert sich in der Politisierung des Konsums und der Selbstinszenierung von Unternehmen als sozial verantwortliche Bürger. Dies wirft grundlegende Fragen zur Neubestimmung von Bürgerschaftskonzepten und zur Erweiterung des Handlungsrepertoires von Protestakteuren in spätmodernen Konsumgesellschaften auf. Dabei fungieren (neue) Medien sowohl als Vermittler unternehmerischen Engagements als auch als Plattform für die Ausbildung neuer Protestformen. Der Band liefert einen Beitrag zur aktuellen Diskussion und versammelt Perspektiven von Wissenschaftlern und Praktikern

Radiation-induced melting in coherent X-ray diffractive imaging at the nanoscale

Coherent X-ray diffraction techniques play an increasingly significant role in imaging nanoscale structures which range from metallic and semiconductor samples to biological objects. The conventional knowledge about radiation damage effects caused by ever higher brilliance X-ray sources has to be critically revised while studying nanostructured materials

Performance and Operation of the CMS Electromagnetic Calorimeter

The operation and general performance of the CMS electromagnetic calorimeter using cosmic-ray muons are described. These muons were recorded after the closure of the CMS detector in late 2008. The calorimeter is made of lead tungstate crystals and the overall status of the 75848 channels corresponding to the barrel and endcap detectors is reported. The stability of crucial operational parameters, such as high voltage, temperature and electronic noise, is summarised and the performance of the light monitoring system is presented