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Cerebrotendinous xanthomatosis (CTX) is a rare autosomal recessive disorder believed to be exclusively caused by mutations in the CYP27A1 gene coding for the enzyme sterol 27-hydroxylase. Common findings in CTX are tendon xanthomas, cataracts and progressive neurological dysfunction. Here, we characterize an adult female patient with tendon xanthomas and classic biochemical findings of CTX (i.e. h

A previously described kinetic model for the Calvin cycle and ancillary pathway of starch production in the chloroplast of C3 plants has been extended so that it becomes applicable under physiological conditions where there is a competition between carbon dioxide and oxygen for ribulose-1,5-bisphosphate carboxylase (rubisco). The modified model is shown to account for the observed dependencies of

The mechanisms involved in the down regulation of the exocrine pancreas are still not fully elucidated. The purpose of the present study was to examine the effect of duodenal and ileal exposure to pancreatic enzymes and bile on pancreatic secretion. Experiments were performed with pigs fitted with pancreatic duct, ileal and jugular vein catheters, and duodenal T-shaped cannula. A negative feedback

The middle Cambrian -Furongian transitional interval was a time of significant biotic and environmental changes. Strata of this age in Scania, southern Sweden, contain two interlayered biofacies, a normal one dominated by trilobites and an anomalous one dominated by phosphatocopines (small bivalved arthropods). In places these biofacies are separated by intervals barren of fossils. In a phosphatoc

The problem of analyzing heart rate variability in the presence of ectopic beats is revisited. Based on the integral pulse frequency modulation model and the closely related heart timing signal, a new technique is introduced which corrects for the occasional presence of ectopic beats. The correction technique, which involves the occurrence times of a certain number of beats preceding the ectopic b

Objectives. To find the time-to-peak for creatine kinase MBmass (CKMB) and cardiac troponin T (cTnT) after acute reperfusion, to compare peak and cumulative values to estimate infarct size (IS), and to evaluate clinical routine sampling for assessment of IS. Design. Acute primary percutaneous coronary intervention (PCI) was performed in 38 patients with first-time myocardial infarction. In 21 pati

1. Mice were subjected to gastrectomy (GX) or food deprivation (24 h). The release of insulin and glucagon in response to different secretagogues was monitored in vivo and in isolated islets 3-4 weeks after surgery. 2. GX animals responded to glucose with an impaired glucose tolerance and a poor increase in plasma insulin. Islets from GX or food-deprived mice displayed impaired insulin release to

This study has determined the fracture mechanical properties of 9 types of rock, namely fine-, medium- and coarse-grained granites, gneiss, quartzite, diabase, gabbro, and fine- and coarse-grained limestones. Test results show among other things that quartzite has the highest compressive strength and fracture energy, while diabase has the highest splitting tensile strength and modulus of elasticit

This article presents research done in order to analyse the machinability of duplex stainless steel with coated carbide tools during turning operations. Although duplex stainless steel has existed for about 70 years fairly little work has been published in the area of investigating the different aspects of the machining of these materials. In this research project three different kinds of duplex s

This paper presents a wideband LC quadrature voltage controlled oscillator (VCO) for novel TRPC-UWB transceiver in 0.13 μm CMOS technology. Based on series coupling structure, and through combining the advanced switched capacitor and MOS varactor techniques, this QVCO's continuous frequency tuning range covers from 5.05 to 5.84 GHz. The measured phase noise at 1 MHz offset from a 5.1 GHz carrier a

Recent research has shown that invariant indexing can speed up the recognition process in computer vision. Extraction of invariant features can be done by choosing first a canonical reference frame, and then features in this reference frame. This paper gives methods for extracting invariants for planar curves under affine and projective transformations. The invariants can be used semilocally to re