Liver failure associated with mitochondrial DNA depletion

BACKGROUND/AIMS: Liver failure in infancy can result from several disorders of the mitochondrial respiratory chain. In some patients, levels of mitochondrial DNA are markedly reduced, a phenomenon referred to as mitochondrial DNA depletion. To facilitate diagnosis of this condition, we have reviewed the clinical and pathological features in five patients with mitochondrial DNA depletion. METHODS: Cases were identified by preparing Southern blots of DNA from muscle and liver, hybridising with appropriate probes and quantifying mitochondrial DNA relative to nuclear DNA. RESULTS: All our patients with mitochondrial DNA depletion died of liver failure. Other problems included hypotonia, hypoglycaemia, neurological abnormalities (including Leigh syndrome) and cataracts. Liver histology showed geographic areas of fatty change, bile duct proliferation, collapse of liver architecture and fibrosis; some cells showed decreased cytochrome oxidase activity. Muscle from three patients showed mitochondrial proliferation, with loss of cytochrome oxidase activity in some fibres but not in others; in these cases, muscle mitochondrial DNA levels were less than 5% of the median control value. The remaining two patients (from a single pedigree) had normal muscle histology and histochemistry associated with less severe depletion of mitochondrial DNA in muscle. CONCLUSIONS: Liver failure is common in patients with mitochondrial DNA depletion. Associated clinical features often include neuromuscular disease. Liver and muscle histology can be helpful in making the diagnosis. Mitochondrial DNA levels should be measured whenever liver failure is thought to have resulted from respiratory chain disease.     

Determination of Turbidity in Kourris Dam in Cyprus Utilizing Landsat TM Remotely Sensed Data

Determination of turbidity is a common component of water-quality assessments. In regions where there are a lot of inland waters such as dams, sampling even a small proportion of those dams for monitoring and assessing water quality is cost prohibitive. Satellite remote sensing has the potential to be a powerful tool for assessing water quality over large spatial scales. The overall objective of this study was to examine whether Landsat-5 TM (Thematic Mapper) and Landsat-7 ETM+ (Enhanced Thematic Mapper) could be used to measure turbidity across theKourris Dam, which is the biggest dam in Cyprus. This paper presents the results obtained by applying the linear regression analysis in order to examine the relationship between the turbidity measurements measured in-situ during the satellite overpass against at-satellite atmospheric corrected reflectance values. It has been found that the reflectance, after atmospheric correction, at LandsatTMBands 1 and 3 is strongly related with turbidity levels after linear regression analysis. The most significant correlation was occurred when reflectance in TM band 3 and logarithmic reflectance in TM band 3 were correlated with turbidity measurements. Indeed, the correlation coefficient (R) when atmospheric corrected reflectance (ρ) in the LandsatTMband 3 were correlated against turbidity, before atmospheric correction was R = 0.38 and after atmospheric correction was R = 1; and when atmospheric corrected logarithmic reflectance (Log ρ) in the Landsat TM band 3 were correlated against turbidity, before atmospheric correction was R = 0.46 and after atmospheric correction was R = 1.     

Did a local distribution company procure prudently during the California electricity crisis?

To manage cost risk, prudent procurement of electric power requires that some portion of a buyer's energy demand be met through long-term contracting. Under cost-of-service regulation or performance-based regulation, a local distribution company (LDC) should be allowed to fully recover all prudently incurred power procurement costs. However, the regulatory test of prudence is an ex post review with the threat of disallowance. This paper presents an economic analysis of procurement prudence involving a small LDC, Bear Valley Electric Service (BVES), which serves a resort area in Southern California. The key findings are: (a) high and volatile prices and rolling blackouts characterized the market environment faced by the owner of BVES, Southern California Water Company (SCWC), at its signing of a 5-year fixed price contract; (b) SCWC was a price-taker with no incentive to act imprudently; (c) the contract was obtained via a competitive bidding process; (d) the contract price was comparable to the benchmark price of contemporaneous contracts; (e) the fixed price contract was economic when compared to available alternatives; and (f) despite (a)–(e), a negotiated settlement with the state regulator and a large user resulted in substantial disallowance. The policy implication is that a regulator should approve a prudent procurement plan proposed by an LDC to remove the unreasonable risk of an ex post review. If the LDC strictly adheres to the plan, the resulting electricity purchases are per se prudent and should entitle the LDC to full cost recovery.     

Memory in food-storing birds: from behaviour to brain

As a result of natural history studies, it has been hypothesized that food-storing birds may develop a special kind of memory to cope with the demand imposed by their food-storing behaviour (i.e. the ability to retrieve food from a wide variety of stores over varying amounts of time after storage). Recent studies on food-storing birds suggest that, at a relatively late stage in their development, the specific memories associated with food-storing behaviour can stimulate growth of the hippocampus, an area of the brain concerned with memory processing.     

Preliminary study on cost optimisation of aircraft composite structures applicable to liquid moulding technologies

This paper details the initial development of a method for determining the associated recurring labour costs for the manufacture of a aircraft component that will form the basis for a computerised methodology for determining the optimum manufacturing method for a component design. The research focuses on the flow of process steps to manufacture an aircraft component for the vacuum-assisted resin transfer moulding and resin transfer moulding manufacturing process. The methodology developed is based on applying MIT cost equations to process steps from which cost variables and constants are established to represent an estimated costing of the aircraft structure. This research will assist in providing a swifter and more accurate conceptual design/manufacturing system that includes an analysis of cost and will assist the production of trade studies that consider the manufacture of aircraft components using cost-effective technologies, such as liquid moulding.     

Combined Experimental and Computational Methods Reveal the Evolution of Buried Interfaces during Synthesis of Ferroelectric Thin Films

Understanding interfaces between dissimilar materials is crucial to the development of modern technologies, for example, semiconductor–dielectric and thermoelectric–semiconductor interfaces in emerging electronic devices. However, the structural characterization of buried interfaces is challenging because many measurement techniques are surface sensitive by design. When interested in interface evolution during synthesis, the experimental challenges multiply and often necessitate in situ techniques. For solution‐derived lead zirconate titanate (PZT) ferroelectric thin films, the evolution of buried interfaces during synthesis (including dielectric–metal and metal–metal) is thought to dramatically influence the resultant dielectric and ferroelectric properties. In the present work, multiple experimental and computational methods are combined to characterize interface evolution during synthesis of ferroelectric PZT films on platinized Si wafers—including in situ X‐ray diffraction during thermal treatment, aberration‐corrected scanning transmission electron microscopy of samples quenched from various synthesis states, and calculations using density functional theory. Substantial interactions at buried interfaces in the PZT/Pt/Ti/SiO _x /Si heterostructure are observed and discussed relative to their role(s) in the synthesis process. The results prove that perovskite PZT nucleates directly from the platinum (111)‐oriented bottom electrode and reveal the roles of Pb and O diffusion and intermetallic Pt₃Pb and Pt₃Ti phases.     

Second generation beatwave experiments at UCLA

The NEPTUNE Laboratory, under construction at UCLA, will be a user facility for exploring concepts useful for advanced accelerators. The primary programmatic goal for the laboratory is to inject extremely high-quality electron bunches into a laser-driven plasma beat wave accelerator and explore ideas for extracting a high-quality ΔE/E < 0.1, < 10 π mm mrad), high-energy (100 MeV) beam from a plasma structure operating at about 1 THz and about 3 GeV/m. The lab will combine an upgraded MARS CO₂ laser and the state-of-the-art SATURNUS RF gun and linac, also undergoing an upgrade. The new MARS laser will be about 1 TW (100 J, 100 ps), up from 0.2 TW (70 J, 350 ps). This allows for doubling the spot size of the laser beam and thereby quadrupling the interaction length while still driving gradients of 3 GeV/m. The large diameter of the accelerating structure relative to the injected electron bunches (10:1 ratio) will minimize the deleterious effects of the radial dependence of the accelerating field and soften the radial focusing thus permitting, in principle, the extraction of a high-quality accelerated beam.     

Genetic nomenclature for Trypanosoma and Leishmania

We investigated the effect of chronic ethanol administration and its withdrawal on the polypeptide levels of NMDA receptor subunits such as NR1, NR2A, and NR2B in the rat cerebral cortex and hippocampus using Western blot analysis technique. Our results indicate that chronic ethanol treatment upregulates NMDA receptor subunits NR1, NR2A, and NR2B ( approximately 35%). At 48 h of last dose of ethanol administration, the protein content returned to almost control level, thereby demonstrating the reversibility of the changes.