Lumican and fibromodulin regulate the assembly of collagens into higher order fibrils in connective tissues. Here, we show that mice deficient in both of these proteoglycans manifest several clinical features of Ehlers-Danlos syndrome. The Lum(-/-)Fmod(-/-) mice are smaller than their wild type littermates and display gait abnormality, joint laxity, and age-dependent osteoarthritis. Misaligned kne

The article may be seen as a sequel to my paper delivered at a conference on art historical subjectivity and methodology in Stockholm 1999, initiated by Margaretha Rossholm Lagerlöf. – Now I will try to move that analysis a little step further by discussing some problems concerning the more explicit performative effects and experiences of everyday technical objects and their occasionally strange v

A capillary-based model modified for characterization of monolithic cryogels is presented with key parameters like the pore size distribution, the tortuosity and the skeleton thickness employed for describing the porous structure characteristics of a cryogel matrix. Laminar flow, liquid dispersion and mass transfer in each capillary are considered and the model is solved numerically by the finite

Lately, sensor networks have received much attention in the research community. One of the aims of these networks is to make them self-configuring and self-healing so that they can be easily deployed and self-sufficient. A fundamental limitation with sensor networks is their limited life span because of their battery operation. To that end, much research has been carried out to maximize the life s

This article proposes Lagrange multiplier-based tests for the null hypothesis of no cointegration. The tests are general enough to allow for heteroskedastic and serially correlated errors, deterministic trends, and a structural break of unknown timing in both the intercept and slope. The limiting distributions of the test statistics are derived, and are found to be invariant not only with respect

The receptor for advanced glycation end products ( RAGE) is a member of the immunoglobulin superfamily being expressed as a cell surface molecule and binding a variety of ligands. One of these ligands is high-mobility group box chromosomal protein 1, a potent proinflammatory cytokine, expression of which is increased in synovial tissue and in synovial fluid of rheumatoid arthritis (RA) patients. T

Aims To explore the morphology of the proximal coronary sinus (CS) and the surrounding tissues in order to identify possible routes for interatrial conduction. Method Specimens containing interatrial septum and proximal CS were taken from 21 necropsied hearts and sliced into 10-mu m thick parallel histological sections in 1-mm steps starting from the valve plane, up to the atrial. roof (40-80 sect

Background & Aims: The long-term prognosis of patients with small-duct primary sclerosing cholangitis (PSC) remains incompletely characterized. We aimed at determining the natural history and long-term outcomes of a large number of patients with small-duct PSC. Methods: Data from 83 patients with well-characterized small-duct PSC from several medical institutions in Europe and the United State

We present a theory for the full distribution of current fluctuations in incoherent diffusive superconducting junctions, subjected to a voltage bias. This theory of full counting statistics of incoherent multiple Andreev reflections is valid for an arbitrary applied voltage. We present a detailed discussion of the properties of the first four cumulants as well as the low and high voltage regimes o

Wheat straw is an abundant agricultural residue which can be used as a raw material for bioethanol production. Due to the high xylan content in wheat straw, fermentation of both xylose and glucose is crucial to meet desired overall yields of ethanol. In the present work a recombinant xylose fermenting strain of Saccharomyces cerevisiae, TMB3400, cultivated aerobically on wheat straw hydrolysate, w

A description of mesoscopic fluctuations of the pairing gap in finite-sized quantum systems based on periodic orbit theory is presented. The size of the fluctuations are found to depend on quite general properties. We distinguish between systems where corresponding classical motion is regular or chaotic, and describe in detail fluctuations of the BCS gap as a function of the size of the system. Th

Bird flight occurs over a range of Reynolds numbers (Re; 104Re105, where Re is a measure of the relative importance of inertia and viscosity) that includes regimes where standard aerofoil performance is difficult to predict, compute or measure, with large performance jumps in response to small changes in geometry or environmental conditions. A comparison of measurements of fixed wing performance a

A theoretical analysis of the microdialysis ethanol technique in skeletal muscle is presented, and a model governing the transport of ethanol from the microdialysis probe to the capillaries in the muscle tissue is proposed. The model is derived under the assumption of a steady-state situation, and an analytical solution is found for the outflow-to-inflow ratio of ethanol in the perfusate. Theoreti

ZnWO4, ZnWO4 : Fe and ZnWO4 : Mo crystals were investigated by the methods of time-resolved spectroscopy in the temperature range of 4.2-300 K. It is shown that the Mo and Fe impurities significantly reduce the light yield of ZnWO4. The main 2.5 eV emission of ZnWO4 and the 1.77 eV emission band of ZnWO4 : Mo are shown to originate from the triplet excited state of the WO6 and MoO6 complex, respec

Scattered vasoactive intestinal polypeptide (VIP)-immunoreactive nerves were found in the striated muscle of the hind limb of the cat, where they usually were associated with small blood vessels. VIP-immunoreactive nerves were also demonstrated in the sciatic nerve; after ligation an abundance of intensely immunoreactive VIP fibres were seen proximal to the ligation. Intraarterial infusion of VIP