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Defective assembly of alpha 3 alpha 4 alpha 5(IV) collagen in the glomerular basement membrane causes Alport syndrome, a hereditary glomerulonephritis progressing to end-stage kidney failure. Assembly of collagen IV chains into heterotrimeric molecules and networks is driven by their noncollagenous (NC1) domains, but the sites encoding the specificity of these interactions are not known. To identi
We report the case of a patient with mild haemophilia A, due to a Tyr2105Cys mutation in exon 22 of the C1 domain, who developed a high-titre factor VIII inhibitor (maximum titre 1600 BU) with recurrent severe haemorrhages and fatal intracranial bleeding. Based on published data, it appears that although this mutation occurs rarely in patients with mild or moderate haemophilia A, it is frequently
The timing of reproduction in birds varies considerably within populations and is often under strong natural selection. Individual timing within years is dependent on a range of environmental factors in addition to having an additive genetic basis. In vertebrates, an increasing amount is known about the molecular basis for variation in biological timing. The Clock gene includes a variable poly-glu
Tailored supercritical carbon dioxide (scCO(2)) extraction of alkyhesorcinols (ARs) from rye bran resulting in pre-purification of ARs already during extraction process. The pre-purification was achieved by splitting the extraction process in 2 steps.. In both steps, the CO2 pressure was set at 25 MPa with a flow rate of 10 g/min. The first step was carried out at 70 degrees C. 0.06% of ethanolic
Scatter and septal penetration deteriorate contrast and quantitative accuracy in single photon emission computed tomography ( SPECT). In this study four different correction techniques for scatter and septal penetration are evaluated for I-123 brain SPECT. One of the methods is a form of model-based compensation which uses the effective source scatter estimation ( ESSE) for modelling scatter, and
We present palaeomagnetic inclinations from Late Cretaceous-Early Tertiary deep-water carbonates obtained during ODP Leg 192 drilling at the Ontong Java Plateau (OJP) for the following periods: magnetochrons C33n (73.6-79.1 Ma), C32 (71.1-73.6 Ma), C27r-C31n (61.3-68.7 Ma) and C25-C26 (55.9-60.9 Ma). Compaction-induced inclination shallowing is considered to be negligible for the OJP sediments exa
New laser stepwise heating Ar-40-Ar-39 dates (415 +/- 1.8 and 414 +/- 2 Ma) from the Etive Dyke Swarm in the SW Grampian Highlands of Scotland indicate that the dykes are 10-20 Ma older than previously assumed. The dykes constitute the final phase of the Late Caledonian Granites in the region, which themselves define the end of the Caledonian Orogeny, therefore these dates can be taken as the end
Treatment with alkali, particularly overliming, has been widely used as a method for the detoxification of lignocellulose hydrolysates prior to ethanolic fermentation. However, the mechanisms behind the detoxification effect and the influence of the choice of cation have not been well understood. In this study, a dilute acid hydrolysate of spruce and an inhibitor cocktail consisting of six known i
This study reports the findings of how district nurses (n = 23) and hospital nurses (n = 9) in cancer care experienced social support in immediate connection with demanding care situations, as well as the effect of systematic clinical supervision. The study is based on their own accounts. The data were collected by means of semi-structured interviews. The findings indicate that there is a great ne
Background: The study was designed to determine whether a 1-year hospital-based secondary prevention programme would have any long-term effects on serum lipid levels and the use of lipid-lowering drugs in patients with coronary artery disease 4 years after referral to primary care facilities for follow-up. Design/methods: After acute myocardial infarction or coronary bypass surgery, 241 consecuti
Purpose Although there is evidence for interaction between epidermal growth factor receptor ( EGFR) and estrogen receptor ( ER), it is still not clear how this affects response to endocrine therapies like tamoxifen. Here we assess the relationship between EGFR expression and tamoxifen response, with a new quantitative technology. Patients and Methods A tissue microarray was constructed from breast
The FRR (Friendly Rest Room) project creates and evaluates prototypes of a more user friendly intelligent toilet for old persons and for persons with disabilities. Additionally, applicable knowledge regarding needs and wishes of old and/or disabled persons and their care persons are documented. In this paper the user driven research approach, ethical aspects and the iterative user centred design p
The phase behavior (temperature vs composition) and microstructure for the two binary systems Pluronic 25R4 [(PO)(19)(EO)33(PO)(19)]-water and Pluronic 25R2 [PO)(21)(EO)(14)(PO)(21)]-water have been studied by a combined experimental approach in the whole concentration range and from 5 to 80 degreesC. The general phase behavior has been identified by inspection under polarized light. Precise phase
Background Treatment of relapsing-remitting multiple sclerosis with interferon beta is only partly effective, and new more effective and safe strategies are needed. Our aim was to assess the efficacy of oral methylprednisolone as an add-on therapy to subcutaneous interferon beta-1a to reduce the yearly relapse rate in patients with relapsing-remitting multiple sclerosis. Methods NORMIMS (NORdic tr
Using a recombinant mini-laminin-332, we showed that truncation of the three C-terminal amino acids of the gamma2 chain, but not of the C-terminal amino acid of the beta3 chain, completely abolished alpha3beta1 integrin binding and its cellular functions, such as attachment and spreading. However, a synthetic peptide mimicking the gamma2 chain C-terminus did not interfere with alpha3beta1 integrin
BACKGROUND: The aim of this study was to investigate, in an experimental survival model, the functional and morphologic results of lung transplantation using lungs from non-heart-beating donors. METHODS: Left lungs, topically cooled to 25 degrees C for 2 hours in situ after 5 minutes of circulatory arrest followed by 26 minutes of unsuccessful cardiopulmonary resuscitation, were transplanted into
The ECG is the key clinical test available for the emergency determination of which patients who presenting with acute coronary syndromes indeed have acute myocardial ischemia/infarction. Because typically the etiology is thrombosis, the correct clinical decision regarding reperfusion therapy is crucial. This review follows the efforts of an AHA working group to develop new standards for clinical
Xylose fermentation by Saccharomyces cerevisiae requires the introduction of a xylose pathway, either similar to that found in the natural xylose-utilizing yeasts Pichia stipitis and Candida shehatae or similar to the bacterial pathway. The use of NAD(P)H-dependent XR and NAD(+)-dependent XDH from P. stipitis creates a cofactor imbalance resulting in xylitol formation. The effect of replacing the
