Inselreich in Bewegung: Durch die Augen einer Journalistin

Journalistin Judith Brandner stellt ihr aktuelles Buch Japan - Inselreich in Bewegung vor und diskutiert über wichtige Zäsuren in der Heisei-Zeit, die sie selbst miterlebt hat. --- Japan has been shaped by dramatic events throughout the past three centuries: the forced opening and the advent of modernity in the 19th century, the first atomic bombs in the 20th century, one of the worst nuclear disasters in history in the 21st century. In her new book, Judith Brandner traces arcs in between these watershed moments. Brandner’s reportages shed light on some of the most serious issues in Japanese society today: fatal demographic trends, the working poor, discussion on Article 9 of the constitution, Okinawans’ fight for independence or the ongoing crisis in Fukushima.Japan has been shaped by dramatic events throughout the past three centuries: the forced opening and the advent of modernity in the 19th century, the first atomic bombs in the 20th century, one of the worst nuclear disasters in history in the 21st century. In her new book, Judith Brandner traces arcs in between these watershed moments. Brandner’s reportages shed light on some of the most serious issues in Japanese society today: fatal demographic trends, the working poor, discussion on Article 9 of the constitution, Okinawans’ fight for independence or the ongoing crisis in Fukushima

Seasonality of bivalve larvae within a high Arctic fjord

Paid Open Acces

The Role of Mass and Environment in Multiple Star Formation: A 2MASS Survey of Wide Multiplicity in Three Young Associations

We present the results of a search for wide binary systems among 783 members of three nearby young associations: Taurus-Auriga, Chamaeleon I, and two subgroups of Upper Scorpius. Near-infrared (JHK) imagery from 2MASS was analyzed to search for wide (1-30"; ~150-4500 AU) companions to known association members, using color-magnitude cuts to reject likely background stars. We identify a total of 131 candidate binary companions with colors consistent with physical association, of which 39 have not been identified previously in the literature. Our results suggest that the wide binary frequency is a function of both mass and environment, with significantly higher frequencies among high-mass stars than lower-mass stars and in the T associations than in the OB association. We discuss the implications for wide binary formation and conclude that the environmental dependence is not a direct result of stellar density or total association mass, but instead might depend on another environmental parameter like the gas temperature. We also analyze the mass ratio distribution as a function of mass and find that it agrees with the distribution for field stars to within the statistical uncertainties. The binary populations in these associations generally follow the empirical mass-maximum separation relation observed for field binaries, but we have found one candidate low-mass system (USco-160611.9-193532; Mtot~0.4 Msun) which has a projected separation (10.8"; 1550 AU) much larger than the suggested limit for its mass. (Abridged)Comment: Accepted to ApJ; 27 pages in emulateapj format. The full version of table 2 can be downloaded via http://www.astro.caltech.edu/~alk/tab2.pdf (PDF) or http://www.astro.caltech.edu/~alk/tab2.txt (text

The driver landscape of sporadic chordoma.

Chordoma is a malignant, often incurable bone tumour showing notochordal differentiation. Here, we defined the somatic driver landscape of 104 cases of sporadic chordoma. We reveal somatic duplications of the notochordal transcription factor brachyury (T) in up to 27% of cases. These variants recapitulate the rearrangement architecture of the pathogenic germline duplications of T that underlie familial chordoma. In addition, we find potentially clinically actionable PI3K signalling mutations in 16% of cases. Intriguingly, one of the most frequently altered genes, mutated exclusively by inactivating mutation, was LYST (10%), which may represent a novel cancer gene in chordoma.Chordoma is a rare often incurable malignant bone tumour. Here, the authors investigate driver mutations of sporadic chordoma in 104 cases, revealing duplications in notochordal transcription factor brachyury (T), PI3K signalling mutations, and mutations in LYST, a potential novel cancer gene in chordoma

Phenotypic heterogeneity and genetic modification of P102L inherited prion disease in an international series

The largest kindred with inherited prion disease P102L, historically Gerstmann-Sträussler-Scheinker syndrome, originates from central England, with émigrés now resident in various parts of the English-speaking world. We have collected data from 84 patients in the large UK kindred and numerous small unrelated pedigrees to investigate phenotypic heterogeneity and modifying factors. This collection represents by far the largest series of P102L patients so far reported. Microsatellite and genealogical analyses of eight separate European kindreds support multiple distinct mutational events at a cytosine-phosphate diester-guanidine dinucleotide mutation hot spot. All of the smaller P102L kindreds were linked to polymorphic human prion protein gene codon 129M and were not connected by genealogy or microsatellite haplotype background to the large kindred or each other. While many present with classical Gerstmann-Sträussler-Scheinker syndrome, a slowly progressive cerebellar ataxia with later onset cognitive impairment, there is remarkable heterogeneity. A subset of patients present with prominent cognitive and psychiatric features and some have met diagnostic criteria for sporadic Creutzfeldt-Jakob disease. We show that polymorphic human prion protein gene codon 129 modifies age at onset: the earliest eight clinical onsets were all MM homozygotes and overall age at onset was 7 years earlier for MM compared with MV heterozygotes (P = 0.02). Unexpectedly, apolipoprotein E4 carriers have a delayed age of onset by 10 years (P = 0.02). We found a preponderance of female patients compared with males (54 females versus 30 males, P = 0.01), which probably relates to ascertainment bias. However, these modifiers had no impact on a semi-quantitative pathological phenotype in 10 autopsied patients. These data allow an appreciation of the range of clinical phenotype, modern imaging and molecular investigation and should inform genetic counselling of at-risk individuals, with the identification of two genetic modifiers

A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea

Kuru is so far the principal human epidemic prion disease. While its incidence has steadily declined since the cessation of its route of transmission, endocannibalism, in Papua New Guinea in the 1950s, the arrival of variant Creutzfeldt–Jakob disease (vCJD), also thought to be transmitted by dietary prion exposure, has given kuru a new global relevance. We investigated all suspected cases of kuru from July 1996 to June 2004 and identified 11 kuru patients. There were four females and seven males, with an age range of 46–63 years at the onset of disease, in marked contrast to the age and sex distribution when kuru was first investigated 50 years ago. We obtained detailed histories of residence and exposure to mortuary feasts and performed serial neurological examination and genetic studies where possible. All patients were born a significant period before the mortuary practice of transumption ceased and their estimated incubation periods in some cases exceeded 50 years. The principal clinical features of kuru in the studied patients showed the same progressive cerebellar syndrome that had been previously described. Two patients showed marked cognitive impairment well before preterminal stages, in contrast to earlier clinical descriptions. In these patients, the mean clinical duration of 17 months was longer than the overall average in kuru but similar to that previously reported for the same age group, and this may relate to the effects of both patient age and PRNP codon 129 genotype. Importantly, no evidence for lymphoreticular colonization with prions, seen uniformly in vCJD, was observed in a patient with kuru at tonsil biopsy

Syntactic architecture and its consequences II: Between syntax and morphology

This volume collects novel contributions to comparative generative linguistics that “rethink” existing approaches to an extensive range of phenomena, domains, and architectural questions in linguistic theory. At the heart of the contributions is the tension between descriptive and explanatory adequacy which has long animated generative linguistics and which continues to grow thanks to the increasing amount and diversity of data available to us. The chapters address research questions in comparative morphosyntax, including the modelling of syntactic categories, relative clauses, and demonstrative systems. Many of these contributions show the influence of research by Ian Roberts and collaborators and give the reader a sense of the lively nature of current discussion of topics in morphosyntax and morphosyntactic variation. This book is complemented by volume I available at https://langsci-press.org/catalog/book/275 and volume III available at https://langsci-press.org/catalog/book/277

Syntactic architecture and its consequences II: Between syntax and morphology

This volume collects novel contributions to comparative generative linguistics that “rethink” existing approaches to an extensive range of phenomena, domains, and architectural questions in linguistic theory. At the heart of the contributions is the tension between descriptive and explanatory adequacy which has long animated generative linguistics and which continues to grow thanks to the increasing amount and diversity of data available to us. The chapters address research questions in comparative morphosyntax, including the modelling of syntactic categories, relative clauses, and demonstrative systems. Many of these contributions show the influence of research by Ian Roberts and collaborators and give the reader a sense of the lively nature of current discussion of topics in morphosyntax and morphosyntactic variation. This book is complemented by volume I available at https://langsci-press.org/catalog/book/275 and volume III available at https://langsci-press.org/catalog/book/277

Syntactic architecture and its consequences II: Between syntax and morphology

This volume collects novel contributions to comparative generative linguistics that “rethink” existing approaches to an extensive range of phenomena, domains, and architectural questions in linguistic theory. At the heart of the contributions is the tension between descriptive and explanatory adequacy which has long animated generative linguistics and which continues to grow thanks to the increasing amount and diversity of data available to us. The chapters address research questions in comparative morphosyntax, including the modelling of syntactic categories, relative clauses, and demonstrative systems. Many of these contributions show the influence of research by Ian Roberts and collaborators and give the reader a sense of the lively nature of current discussion of topics in morphosyntax and morphosyntactic variation. This book is complemented by volume I available at https://langsci-press.org/catalog/book/275 and volume III available at https://langsci-press.org/catalog/book/277

Syntactic architecture and its consequences II: Between syntax and morphology

This volume collects novel contributions to comparative generative linguistics that “rethink” existing approaches to an extensive range of phenomena, domains, and architectural questions in linguistic theory. At the heart of the contributions is the tension between descriptive and explanatory adequacy which has long animated generative linguistics and which continues to grow thanks to the increasing amount and diversity of data available to us. The chapters address research questions in comparative morphosyntax, including the modelling of syntactic categories, relative clauses, and demonstrative systems. Many of these contributions show the influence of research by Ian Roberts and collaborators and give the reader a sense of the lively nature of current discussion of topics in morphosyntax and morphosyntactic variation. This book is complemented by volume I available at https://langsci-press.org/catalog/book/275 and volume III available at https://langsci-press.org/catalog/book/277