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Retinal cone photoreceptors mediate fine visual acuity, daylight vision, and color vision. Congenital hereditary conditions in which there is a lack of cone function in humans cause achromatopsia, an autosomal recessive trait, characterized by low vision, photophobia, and lack of color discrimination. Herein we report the identification of mutations in the PDE6C gene encoding the catalytic subunit

The coexistence of two lamellar liquid crystalline phases has been investigated by means of Monte Carlo simulations. The surfaces of the negatively charged bilayers formed by the surfactant molecules are modeled as planar infinite walls with a uniform surface charge density. Water is treated as a dielectric continuum, and only electrostatic interactions are considered. The counterions are mono- an

For years, safety improvements have been made by evaluating incident reports and analyzing errors and violations. Current developments in safety science, however, challenge the idea that safety can meaning-fully be be seen as the absence of errors or other negatives. Instead, the question becomes whether a company is aware of positive ways in which people, at all level of the organisation, contrib

Although no consensus on the exact dosing schedule of prophylaxis in non-inhibitor haemophilia patients has been achieved, regular administration of factor VIII (FVIII) or factor IX (FIX) concentrates is currently accepted as the most effective treatment model in order to prevent sick days, hospital stays and the development of arthropathy in patients with severe haemophilia. The basis for prophyl

PURPOSE: Malignant fibrous histiocytoma (MFH) has been regarded as the most common soft tissue sarcoma (STS) in adults. Yet its true nature and the validity of this diagnostic concept have increasingly been questioned. Available data suggest that most patients with MFH can be subclassified into specific STS types, but the clinical relevance of such categorization has been argued. In a retrospectiv

GaAs containing buried nanoscale tungsten particles has been characterized electrically. The particles were produced using a special aerosol process and were embedded in GaAs by epitaxial overgrowth. Two different particle sizes were investigated separately. When the particle concentration was increased, a conductance drop of about 500 times was observed. A simulation model, based on a random dist

The solution-phase assembly of 15 nm gold particles into relatively linear chains of fairly controllable length of up to 1 mum is achieved by molecularly linking nanoparticles with alkanedithiols. This step-growth process can be controlled to prepare dimers, oligomers, and polymer-like gold nanoparticle chains by varying the ratio of alkanedithiols to nanoparticles. These size-controlled, relative

Aim: The main aim of the study was to describe the differences between some Northern countries regarding what foods, according to the patients, elicit hypersensitivity symptoms. Methods: At the participating clinics, patients with a history of food hypersensitivity (n = 1139) were asked to fill in a questionnaire in which 86 different foodstuffs were listed. Skin-prick tests (SPT) were performed w

A liquid-cell-based cantilever sensor system operating in the dynamic mode has been developed and characterized, and the frequency spectra of commercial micrometre-scale silicon cantilevers in aqueous solutions have been studied. We report data demonstrating measurements of the resonance frequency shift induced upon phospholipid vesicle adsorption on an oscillating cantilever immersed in a liquid.

OBJECTIVES: The effects of occupational and leisure-time exposures on the risk of acute myeloid leukemia (AML) were investigated with emphasis on clonal chromosome aberrations (CCA) and morphological subtypes. METHODS: Consecutively diagnosed cases of AML (N=333) and 1 population referent per case were retrospectively included in the study. Information on worktasks, companies, and leisure-time act

A computer program for preliminary cost estimates of free and immobilized enzyme systems has been developed. The cost for the hydrolysis of lactose by β-galactosidase fromAspergillus oryzae has been calculated for a batch tank reactor, with free (BTRF) and immobilized (BTRI) enzymes, a continuously stirred tank reactor (CSTR) and a plug-flow tubular reactor (PFTR), considering the mass transfer be

The targeting mechanisms for granule proteins in hematopoietic cells are largely unknown. Aggregation is believed to be important for protein sorting-for-entry and sorting-by-retention in endocrine and neuroendocrine cells. We asked whether artificially induced multimerization/aggregation of chimeric proteins could affect their sorting in hematopoietic cells. A system was used that permits ligand-

Housing rats in an enriched environment improves functional outcome after ischemic stroke, this may reflect neuronal plasticity in brain regions outside the lesion. Which components of the enriched environment that are of greatest importance for recovery after brain ischemia is uncertain. We have previously found that enriched environment and social interaction alone both improve functional recove

NMR spin relaxation in the rotating frame (R-1 rho) is a unique method for atomic-resolution characterization of conformational ( chemical) exchange processes occurring on the microsecond time scale. Here, we use amide H-1 off-resonance R-1 rho relaxation experiments to determine exchange parameters for processes that are significantly faster than those that can be probed using N-15 or C-13 relaxa