Deficiencies in a tyrosine kinase, designated Btk, cause X-linked agammaglobulinemia (XLA) in man, a hereditary defect of B-cell differentiation. Mutations in the newly found PH domain located at the N-terminus of Btk have been shown to be the direct cause of XLA, and here two new mutations, T33P and V64F, are presented. Btk is thus far the only protein in which mutations of the PH domain have bee

PEG-12-acyloxystearates constitute a novel class of pharmaceutical solubilizers and are synthesized from polyethylene glycol and 12-hydroxystearic acid, which has been esterified with a second acyl chain. The hemolytic activity of these surfactants decreases drastically with increasing pendant acyloxy chain length, and surfactants with an acyloxy chain of 14 carbon atoms or more are essentially no

By using bootstrap technique we investigate the properties of the Breusch [Breusch, T.S., 1978. Testing for autocorrelation in dynamic linear models. Australian Economic Papers 17, 334-355]-Godfrey [Godfrey, L.G., 1978. Testing for higher order serial correlation in regression equations when the regressors include lagged dependent variables. Econometrica 46, 1303-1310] autocorrelation tests in dyn

Customer orientation is strongly visible in the visions and strategies of most organizations, but how do these visions and strategies move from intentions to practice? This question provides the focus for this research which aims to acquire deeper insights into this process. The point of departure is the change in perspective from a product to a customer orientation in product and service developi

Streptococcus pyogenes secretes a specific immunoglobulin G (IgG)-protease, SpeB, as well as the IgG glycan-hydrolyzing enzyme EndoS. Here we show that SpeB also degrades IgA, IgM, IgD, and IgE. We also show that EndoS only hydrolyzes the glycan moiety on native but not denatured IgG. Thus, SpeB has a broad immunoglobulin-degrading activity, while EndoS is highly specific for IgG.

Dynamic electric (DEA) and mechanical (DMA) analyses were combined to explore the relaxational processes in amorphous and semicrystalline poly(ethylene naphthalene-2,6-dicarboxylate) (PEN) samples. Differential scanning calorimetry measurements were carried out to investigate the crystallinity of the samples following isothermal annealing treatment at 443 K. The two secondary relaxations beta and

Periodic signals can be decomposed into sets of sinusoids having frequencies that are integer multiples of a fundamental frequency. The problem of finding such fundamental frequencies from noisy observations is important in many speech and audio applications, where it is commonly referred to as pitch estimation. These applications include analysis, compression, separation, enhancement, automatic t

Within the series of articles investigating the burden of rheumatoid arthritis (RA), this paper reviews the methods used for economic assessment of the RA treatments by HTA agencies and other bodies involved in cost-effectiveness analysis and the current status of the field. The overall methods, as well as the challenges, of cost-effectiveness analysis in RA are common to all chronic progressive d

Atomic (1 Å) resolution x-ray structures of horse liver alcohol dehydrogenase in complex with NADH revealed the formation of an adduct in the active site between a metal-bound water and NADH. Furthermore, a pronounced distortion of the pyridine ring of NADH was observed. A series of quantum chemical calculations on the water-nicotinamide adduct showed that the puckering of the pyridine ring in the

Haploid hybrid gametophytes are often present at low frequencies in sympatric populations of Sphagnum capillifolium and Sphagnum quinquefarium. We used intersimple sequence repeat (ISSR) markers and polymerase chain reaction-restriction fragment length polymorphism of the trnL(UAA) intron of the chloroplast genome to reveal the nuclear and chloroplast composition of mature hybrid gametophytes from

Speciation and equilibria of 1,2,4,5-benzenetetracarboxylate (pyromellitate) at the water-boehmite (gamma-AlOOH) interface were studied in 0.1 M Na(Cl) at 298 K. Surface equilibrium analyses were based upon potentiometric and adsorption measurements in the range 4.5 < pH < 10.0. To characterize the surface complexes at the molecular level, attenuated total reflectance infrared spectra were recorde

Background: Autologous platelet rich plasma (PRP) harvest with autotransfusion devices has been used for 10 years in cardiac surgery and recently in orthopedics as a blood saving method. The quality of the harvested platelets has not been adequately examined, in part because of methodological difficulties in studying platelet function during surgery. Methods: Twenty patients undergoing primary to

Researchers examining self-esteem functioning typically consider self-esteem either an affective or competence related trait. However, to capture more precisely the role of self-esteem in behavior we need to view self-esteem both as something people hold and as something they strive for, and determine the relation between these aspects. In the present study the consequences of the dynamic interpla

Background: Caloramator celer is a strict anaerobic, alkalitolerant, thermophilic bacterium capable of converting glucose to hydrogen (H2), carbon dioxide, acetate, ethanol and formate by a mixed acid fermentation. Depending on the growth conditions C. celer can produce H2 at high yields. For a biotechnological exploitation of this bacterium for H2 production it is crucial to understand the factor

I den norska författaren Hanne Ørstaviks sjunde roman Prästen från 2004 förflyttas läsaren till Nordens yttersta utposter. Berättarjaget, vid namn Liv, har som en följd av ett tragiskt händelseförlopp avbrutit sina doktorandstudier i systematisk teologi i södra Tyskland. Istället har hon förpassat sig själv till den karga Finnmarken, där hon nu verkar som präst och febrilt brottas med att ge liv å

The study purpose was to evaluate sleep structure following Vagus Nerve Stimulation (VNS) in 15 children with therapy resistant epilepsy and to correlate possible alterations with changes in epileptiform activity and clinical effects. Fifteen children were examined with ambulatory polysomnographic recordings initially, and after 3 and 9 months of VNS-treatment. Sleep parameters, all-night delta po

We have developed a new Internet service, which provides mobile access to bioinformatics databases and software tools. The BioWAP service facilitates access to basic bioinformatics databases and analysis tools from everywhere without a PC or a laptop computer. Both open source bioinformatics. program suites and Internet services, which are not designed for mobile Internet access, were utilized in

The present investigation addresses mazes in Swedish pre-school children with specific language impairment (SLI). Spontaneous speech samples from 10 Swedish children were collected and analysed with respect to amount of mazes and their distributional patterns. The subjects consisted of five children with SLI and five MLU (mean length of utterance) matched controls with normal grammatical developme

We calculate level densities and pairing gaps for an ultracold dilute gas of fermionic atoms in harmonic traps under the influence of mean field and anharmonic quartic trap potentials. Supershell nodes, which were found in Hartree-Fock calculations, are calculated analytically within periodic orbit theory as well as from WKB calculations. For attractive interactions, the underlying level densities

Haptoglobin phenotypes have been shown in human medicine to be related to the prevalence of various diseases. Furthermore, abnormal glycosylation of haptoglobin has been reported as a consequence of liver disease, cancer and immunological disorders in man. To our knowledge, similar findings have not, so far, been reported in canine disease. The present paper describes a method for investigation of