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Hypophosphatasia is an inherited disorder characterized by defective bone mineralization and deficiency of serum and tissue liver/bone/kidney alkaline phosphatase (L/B/K ALP) activity. We report the characterization of ALPL gene mutations in a series of 11 families from various origins affected by perinatal and infantile hypophosphatasia. Sixteen distinct mutations were found, fifteen of them not
Eight cold-adapted, polygalacturonase-producing yeasts belonging to four species were isolated from frozen environmental samples in Iceland. They were identified as Cystofilobasidium lari-marini, Cystofilobasidium capitatum, Cryptococcus macerans and Cryptococcus aquaticus species by sequence analysis of rDNA regions. Growth behavior of the isolates was investigated. All strains could grow at 2°C.
Neuroblastoma is derived from the sympathetic nervous system and might arise as a result of impaired differentiation, retaining the neuroblastic tumor cells in the cell cycle. Thus, to understand the genesis of neuroblastoma, the study of mechanisms and genes regulating normal sympathetic development is of potential interest. The basic helix-loop-helix transcription factors human achaete-scute hom
To find out whether IgG genes are involved in atopy we studied 26 of 101 laboratory technicians who had developed laboratory animal allergy (LAA). The genes for the constant region of the heavy chains of IgG subclasses were analyzed by serum Gm allotypes, representing products on allelic level of the IGHCG1, IGHCG2 and IGHCG3 on chromosome 14q32. There was a significantly increased frequency of th
Background and purpose Vascular dementia (VaD) has occasionally been associated with cerebral amyloid angiopathy (CAA), but the prevalence and significance of this counterintuitive relationship are poorly known. Therefore, we investigated the presence and characteristics of CAA in brains of VaD cases. Methods We examined temporal and parietal regions of the cerebral cortex of 26 consecutive VaD ca
For many applications, nanoparticles from the gas phase are of interest due to their physical properties. Especially for electronic or optoelectronic applications, the transfer from their random distribution in the gas phase onto flat. substrate surfaces has to be controlled because the particles are needed in exactly defined areas on the substrate. We demonstrate a parallel process for the transf
Kraft black liquor lignin is a biofuel that is separated from the cellulose during kraft pulping. Improved technology and energy integration in paper mills have led to an energy surplus at many mills. It is therefore of great interest to extract the lignin from the pulp mill and sell it as fuel to replace fossil fuel in other furnaces. The fractionation of kraft black liquor with a total dry matte
Clozapine has recently been associated with venous thromboembolism. The aim of this study was to describe three elderly patients in whom olanzapine therapy was associated with venous thromboembolism. During a 4-month period at the same psychogeriatric clinic, three elderly patients (an 89-year-old male, a 78-year-old male and an 83-year-old female) developed deep venous thrombosis shortly after tr
Learning of classically conditioned eyeblink responses depends on mechanisms within the cerebellum. It has been suggested that climbing fibres from the inferior olive transmit the unconditioned stimulus signal to the cerebellum. We have previously shown that the pathway from the deep cerebellar nuclei to the inferior olive inhibits olivary activity. It is known that repeated presentation of the co
Abstract. In this paper we examine subalgebras on two generators in the univariate polynomial ring. A set, S, of polynomials in a subalgebra of a polynomial ring is called a canonical basis (also referred to as SAGBI basis) for the subalgebra if all lead monomials in the subalgebra are products of lead monomials of polynomials in S. In this paper we prove that a pair of polynomials ff; gg is a can
Although the biological properties of staphylococcal enterotoxin A (SEA) have been well characterized, structural insights into the interaction between SEA and major histocompatibilty complex (MHC) class II have only been obtained by modeling. Here, the crystal structure of the D227A variant of SEA in complex with human MHC class II has been determined by X-ray crystallography. SEA(D227A) exclusiv
The uniaxial chain alignment and equatorial patterning of poly [9,9-bis(2-ethylhexyl)fluorene-2,7-diyl] (M-n = 29 kg/mol, M-w = 68 kg/mol) thin films atop rubbed polyimide substrates have been studied by grazing incidence X-ray diffraction. This specific molecular weight yields among the highest observed levels of chain alignment and optical anisotropy and, after thermal annealing, the sample unde
Four different ways to detect Kraft and soluble pine lignin using either soluble or immobilised Trametes hirsuta laccase have been investigated. In the first, the concentration of lignin was measured using laccase in homogeneous medium with a Clark electrode. In the second, laccase immobilised onto aminated porous glass beads was used in a reactor submerged into the lignin containing solution and
A chip for digital intermediate frequency filtering is introduced. The filter is intended move most of the analog intermediate frequency filtering to the digital domain in systems like the American mobile radio system (IS-54). It is a wave digital lattice filter realized with bit-serial arithmetic. Furthermore, a technique for local clocks on chip is presented. The method is based on a ring oscill
An external device ("rottometer") specially designed to measure knee joint rotation was developed and evaluated with respect to its validity. Simultaneous measurements were made with the rottometer and Roentgen stereometric analysis (RSA) in five patients with implanted tantalum markers in the tibia and femur. Measurements of internal and external rotation were made at 90 degrees and 60 degrees of
We report experimental oscillator strengths for 88 Ti I transitions covering the wavelength range 465-3892 nm, 67 of which had no previous experimental values. Radiative lifetimes for 13 energy levels, including the low energy levels 3d(2)(F-3) 4s4p ((3)p) z(5)D(j)degrees have been measured using time-resolved laser-induced fluorescence. Intensity-calibrated Ti I spectra have been measured using F
