Copper and zinc metabolism in aspartylglycosaminuria and Salla disease

Changes in the metabolism of copper and zinc are described in aspartylglycosaminuria (AGU) patients. AGU patients had significantly reduced serum zinc concentrations. However, hair zinc levels were normal, and hyperzincuria could not be demonstrated. The copper content in the hair of AGU patients was highly elevated. Serum copper and ceruloplasmin concentrations were within normal range. In AGU, small-molecular-weight glycoasparagine storage products accumulate in tissues and are excreted in urine in large amounts. They may interfere with the transport mechanisms of trace elements, and thus alter their distribution and availability for tissues. The changes in copper and zinc levels may contribute to the pathogenesis of some of the clinical signs of AGU and Salla disease.     

The distribution of nutrient elements in cell wall and other fractions of the herbage of some grasses and legumes

The distribution of 10 nutrient elements was assessed in three fractions of herbage samples of perennial ryegrass, tall fescue, white clover and lucerne. The cell wall fraction, prepared by mechanical disintegration of undried material, contained the following proportions of the total herbage content of the major elements: N, 2.9–16.4%; P, 3.0–6.9%; S, 3.6–17.2%; Ca, 11.3–51.8%; Mg. 6.4–27.6% and K, 0.1–1.7%. Substantial though more variable proportions of the trace element cations, Fe, Mn, Zn and Cu were also present in the cell wall fraction. The extent to which the elements were soluble in water was assessed by analysis of the water-insoluble fraction prepared from freeze-dried samples of each herbage. The proportions of the total herbage content of the major elements soluble in water were: N, 30–57%; P, 80–92%; S, 35–71%; Ca, 48–69%; Mg. 65–83% and K, 90–96%. With the trace element cations the proportions soluble in water were more variable, though they were generally less than 70% of the total. The extent to which the various elements were soluble in 80% aqueous ethanol was assessed by analysis of the insoluble fraction prepared from freeze-dried materials. With each of the major elements, smaller proportions of the total herbage content were soluble in 80% ethanol than in water, which may reflect in part the lower solubility of proteins and pectin. With the trace element cations, the differences in solubility between 80% ethanol and water were generally small and inconsistent.     

Distribution of diethylstilboestrol in pigs

The synthetic growth promoter diethylstilboestrol (DES) administered orally to pigs can be detected by radioimmunoassay (RIA) analysis of kidney, liver, faeces, bile and urine from animals fed continuously to slaughter, but not in muscle, fat or plasma. If treated animals are fed on material not containing DES for 72 h prior to slaughter, then the levels of parent compound and metabolite in all products decrease to become not significantly greater than those in control animals. The gross metabolism of DES in pigs and bovines appears to be similar.     

Putting Web Services in Context

When considering the full range of Web service-related activities, it becomes clear that dealing with context is a major challenge, requiring greater expressiveness, reasoning capabilities, and architectural components than are provided by the current widely accepted building blocks of the Web services stack. This paper presents an informal overview of concepts, requirements and challenges for handling contextual knowledge in connection with Web services, and briefly discusses several interesting projects in this area of research.     

Transport and energy in developing countries

The author examines the transport energy consumption of developing countries and compares it with that of developed countries. These figures are found to suggest considerable scope for transport energy savings. Given the need for improved energy efficiency, the author moves on to consider transport in the context of the wider economy. Four broad policy approaches which can affect transport energy consumption are identified — information and training programmes, subsidies; pricing and tax policies; and administrative regulations. The author's primary conclusion is that the energy price changes of the last decade do not hold revolutionary implications for the transport sector. There remains, however, a need to improve efficiency in this sector.     

A Comparison of Presentation Methods for Reading on Mobile Phones

A study compares two methods of reading text-based content on mobile phones: traditional scroll-based reading and Rapid Serial Visual Presentation, which displays words rapidly in a sequence. University students used a prototype called Feedo to test both methods, and their reading comprehension, efficiency, and preference ratings were measured. The results show that efficiency increases with fast RSVP, comprehension is equal, and preference rating is lower than with self-paced scroll.     

Prevalence of serologic markers of HBV, HDV, HCV and HIV in non-injection drug users compared to injection drug users in Gran Canaria, Spain

Von Hippel-Lindau disease (VHL) is an autosomal dominant tumour syndrome caused by germline mutations of the VHL tumour suppressor gene located on chromosome 3p25-26. In VHL tumours may occur in 14 different target organs, including the eye. Retinal angiomas are considered the first manifestation of VHL disease in 43% of cases, and the cumulative probability of developing a retinal angioma in one or both eyes rises during each decade of life, reaching 80% for patients over 80 years old. Since 1976 patients with VHL at the University Hospital of Utrecht and their at-risk relatives have been screened periodically by a multidisciplinary team. Long-term follow-up ophthalmological data were analysed with special attention to natural course and results of treatment. In addition, we looked for a genotype-phenotype correlation. Retinal angiomas were found in all families. In one large family with a missense mutation (V170D) of the VHL gene, in which the complete spectrum of visceral- and central nervous system (CNS) features of VHL is present, macular, parapapillary, optic disc and ora serrata angiomas were also found. In general, however, a clear-cut genotype-phenotype correlation could not be found. Only early detection and treatment of peripheral retinal angiomas can be expected to decrease the percentage of patients with decreased visual acuity. Therefore, early detection and treatment of these tumours is of paramount importance. Ophthalmological screening of patients and persons at risk should start as early as possible. In patients with apparently sporadic retinal angiomas it is advisable to perform germline DNA analysis, since the risk of developing VHL is high, especially if the angiomas are bilateral, or unilateral and multifocal, if the patient is young, or if there is a family history suggestive of VHL.