Anamestic inhibitors represent the major complication of haemophilia therapy now that clotting factor concentrates are virtually free of pathogen-transmission risk. Conventional clotting factor replacement is usually insufficient to prevent or treat bleeding in a haemophilia patient with a high responding inhibitor so that alternative treatment with bypassing agents is required. Despite their rela

In this paper, we describe a concise method for the feature-based representation of regions in an indoor environment and show how it can also be applied for door-passage-independent detection of transitions between regions to improve communication with a human user.

Background: The L68Q variant of cystatin C is highly amyloidogenic forming aggregates in individuals with HCCAA. Results: Spermatozoa from mice expressing human L68Q cystatin C exhibit fertility defects and increased levels of amyloid. Conclusion: L68Q epididymal fluid containing cystatin C amyloid is harmful for sperm function. Significance: Amyloid in the reproductive tract may contribute to mal

We identify a mutation (D262N) in the erythroid-affiliated transcriptional repressor GFI1B, in an acute myeloid leukemia (AML) patient with antecedent myelodysplastic syndrome (MDS). The GFI1B-D262N mutant functionally antagonizes the transcriptional activity of wild-type GFI1B. GFI1B-D262N promoted myelomonocytic versus erythroid output from primary human hematopoietic precursors and enhanced cel

Multisensory contributions to perception are well studied, but their underlying brain mechanisms are poorly understood. A new study has exploited advances in fly optogenetics to pinpoint mechanisms that enhance responses to visual motion in the presence of ecologically relevant odors.

A search is presented for production of a heavy up-type quark (t') together with its antiparticle, assuming subsequent decay to a W boson and a b quark, t'(t) over bar' -> W(+)bW(-)(b) over bar. The search is based on 1.04 fb(-1) of proton-proton collisions at root s = 7 TeV collected by the ATLAS detector at the CERN Large Hadron Collider. Data are analyzed in the lepton + jets final state, chara

Machine strength grading of structural timber is based upon relationships between so called indicating properties (IPs) and bending strength. However, such relationships applied on the market today are rather poor. In this paper, new IPs and a new grading method resulting in more precise strength predictions are presented. The local fibre orientation on face and edge surfaces of wooden boards was

The structure of quasi-2d solutions of dipolar superballs in the fluid state has been determined by Metropolis Monte Carlos simulations in the absence and the presence of an external field. Superballs are 3d objects characterized by a one shape parameter. Here, superballs resembling cubes, but possessing rounded edges, have been used. Examination has been made for several magnitudes of the dipole

In this article we demonstrate how both state structures and collective agencies contribute to patterns of securitization and, in so doing, reconfigure conceptions of space and place. Focusing on the life-chances of Muslim minority populations in Denmark, Sweden, and Canada, we begin by establishing how experiences of empire and colonization have shaped dominant regimes of citizenship and multicul

The greatest barrier to successful haemophilia A care in resource-rich countries is the development of inhibitors to therapeutic factor VIII. Children with inhibitors suffer through increased bleeding and joint damage as well as frequent venepuncture. Costs associated with inhibitors are beyond many healthcare systems. Over the last two decades, there has been no improvement in our ability to redu

We present temperature dependent electrical measurements on n-type InAs/InSb nanowire heterostructure field-effect transistors. The barrier height of the heterostructure junction is determined to be 220 meV, indicating a broken bandgap alignment. A clear asymmetry is observed when applying a bias to either the InAs or the InSb side of the junction. Impact ionization and band-to-band tunneling is m

Nitrogen (N) added through atmospheric deposition or as fertilizer to boreal and temperate forests reduces both soil decomposer activity (heterotrophic respiration) and the activity of roots and mycorrhizal fungi (autotrophic respiration). However, these negative effects have been found in studies that applied relatively high levels of N, whereas the responses to ambient atmospheric N deposition r

Early-onset dominantly inherited forms of Alzheimer's disease (AD) are rare, but studies of such cases have revealed important information about the disease mechanisms. Importantly, mutations in amyloid precursor protein (APP), presenilin 1 (PSEN1) and PSEN2, alter the APP processing and lead to an increased amyloid beta-peptide (A beta) 42/40 ratio. This, together with other studies on pathogenic

Most known starfish species possess a compound eye at the tip of each arm, which, except for the lack of true optics, resembles an arthropod compound eye. Although these compound eyes have been known for about two centuries, no visually guided behaviour has ever been directly associated with their presence. There are indications that they are involved in negative phototaxis but this may also be go

In southern African Iron Age studies, there are few attempts to systematically apply and include laboratory analyses when studying archaeological ceramic materials. As demonstrated in this paper, such analyses help to understand technological aspects such as raw materials, manufacturing techniques and vessel function. Combined with vessel shape and decoration as well as ethnographic studies the re

INTRODUCTION: Structural right atrial abnormalities have been described in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). However, little is known about electrocardiographic signs of atrial involvement in ARVC because no systematic studies have been conducted. METHODS: P-wave-triggered signal-averaged orthogonal electrocardiogram from 40 ARVC patients (46 ± 15 years, 16 fema