GRACILE (growth retardation, aminoaciduria, cholestasis, iron overload, lactacidosis, and early death) syndrome is a recessively inherited lethal disease characterized by fetal growth retardation, lactic acidosis, aminoaciduria, cholestasis, and abnormalities in iron metabolism. We previously localized the causative gene to a 1.5-cM region on chromosome 2q33-37. In the present study, we report the

Methyl cellulose (MC) was partially depolymerised and the oligomers thus obtained were studied by matrix-assisted laser desorption/ionisation time-of-flight mass spectrometry (MALDI-TOFMS). The depolymerisation was either enzymatic or acidic. Fractions of enzymatically depolymerised MC were collected from size-exclusion chromatography and subjected to a sample preparation investigation. Several MA

This article is concerned with the relationship between generalised and particularistic knowledge in the context of policy-making and policy analysis. It argues that it is problematic to assume that a reform model will generate similar outcomes across a wide variety of contexts. It presents a conceptual framework, including the concepts of transaction domain and domain consensus, that enables cont

The Goto-Kakizaki (GK) rat displays a markedly reduced insulin response to glucose, a defect that is thought to be coupled to an impaired glucose signaling in the beta-cell. We have examined whether carbon monoxide (CO), derived from beta-cell heme oxygenase (HO), might be involved in the secretory dysfunction. Immunocytochemical labeling of constitutive HO (HO-2) showed no overt difference in flu

ProteomeBinders is a new European consortium aiming to establish a comprehensive resource of well-characterized affinity reagents, including but not limited to antibodies, for analysis of the human proteome. Given the huge diversity of the proteome, the scale of the project is potentially immense but nevertheless feasible in the context of a pan-European or even worldwide coordination.

Rats were made hypo- or hyperthyroid to study the role of thyroid hormones on cerebral transthyretin (TTR) mRNA expression. TTR mRNA was detected by Northern blot in rat liver, choroid plexus and meninges but not in cultured astrocytes or cultured cerebral endothelial cells. No changes were found in the levels of TTR mRNA in liver, choroid plexus or meninges in hypo- or hyperthyroid rats compared

The effective population size (N-e), and the ratio between N-e and census population size (N) are often used as measures of population viability. We show that using the harmonic mean of population sizes over time - a common proxy for N-e- has some important evolutionary consequences and implications for conservation management. This stems from the fact that there is no unambiguous relationship bet

OBJECTIVE: to evaluate dimensions of both parents' postnatal sense of security the first week after childbirth, and to determine associations between the PPSS instrument and different sociodemographic and situational background variables. DESIGN: evaluative, cross-sectional design. PARTICIPANTS AND SETTINGS: 113 mothers and 99 fathers with children live born at term, from five hospitals in souther

Abstract in UndeterminedThe effect of experimental acidification on the soil microfungalcommunity was studied in the humus layer of a coniferous forest in northern Sweden. The study was made 4 years after the last application of sulfuric acid. Fungal species composition was altered by treatments of 100 and 150 kg sulfuric acid ha-1 each year for 6 years, yet no differences were found between the c

Objective To review publications regarding possible donation of surplus embryos for medical research. Design Review of 67 scientific publications in this field up through January 2007. Originality Summary of factors influencing prospective donors' motivation to donate or not donate their surplus embryos for medical research. Result(s) Presentation of the findings of studies regarding the need

Two short (< 2 s) gamma-ray bursts (GRBs) have recently been localized(1-4) and fading afterglow counterparts detected(2-4). The combination of these two results left unclear the nature of the host galaxies of the bursts, because one was a star-forming dwarf, while the other was probably an elliptical galaxy. Here we report the X-ray localization of a short burst (GRB 050724) with unusual gamma-ra

Shredded straw of Miscanthus was composted in 800-L boxes with different amounts of pig slurry added as nitrogen source. The impact of the different initial CIN ratios (11, 35, 47, 50, and 54) on the composting process and the end product was evaluated by examining chemical and microbiological parameters during 12 months of composting. Low initial C/N ratios caused a fast degradation of fibers dur

1. Recently, there has been much interest in physiological trade-offs between parasite resistance and fitness-related traits such as secondary sexual characters or reproductive effort. More specifically it has been suggested that (i) energetically costly activities may suppress the immune system and (ii) that this immunosuppression is caused by costly immune defences competing with other bodily de

OBJECTIVE: To assess the frequency of epilepsy in primary and secondary antiphospholipid syndrome (APS); to analyze the clinical and laboratory features characterizing those with epilepsy in a cohort of 538 patients with APS; and to find associated features that would suggest risk factors for epilepsy in APS. METHODS: We analyzed the clinical features of patients with APS who had epilepsy and comp

The ability of poly(ethylene oxide)-poly(propylene oxide)-poly (ethylene oxide) (PEO-PPO-PEO) block copolymers (Poloxamers) to form "gels" (lyotropic liquid crystalline structures) in water is of interest to pharmaceutical applications. In such applications the presence of polar organic solvents is often desirable or required. The effect of such solvents on the stability of lyotropic liquid crysta

Sugarcane bagasse was pre-treated by steam explosion at 205 and 215degreesC and hydrolysed with cellulolytic enzymes. The hydrolysates were subjected to enzymatic detoxification by treatment with the phenoloxidase laccase and to chemical detoxification by overliming. Approximately 80% of the phenolic compounds were specifically removed by the laccase treatment. Overliming partially removed the phe