Deficit

Mucopolysaccharidosis III (MPS III) is characterized by lysosomal accumulation of the glycosaminoglycan (GAG) heparan sulphate (HS). In humans, the disease manifests in early childhood, and is characterized by a progressive central neuropathy leading to death in the second decade. This disease has also been described in mice (MPS IIIA and IIIB), dogs (MPS IIIA), emus (MPS IIIB) and goats (MPS IIID). We now report on dogs with naturally occurring MPS IIIB, detailing the clinical signs, diagnosis, histopathology, tissue enzymology and substrate levels. Two 3-year-old Schipperke dogs were evaluated for tremors and episodes of stumbling. Examination of the animals found signs consistent with cerebellar disease including dysmetria, hind limb ataxia and a wide-based stance with truncal swaying. There were mildly dystrophic corneas and small peripheral foci of retinal degeneration. Magnetic resonance imaging of the brain and skeletal radiographs were normal. Intracytoplasmic granules were ...

The visual processing of text occurs spontaneously in most readers. Dyslexic persons, however, often report both somatic symptoms and perceptual distortions when trying to read. It is possible that the perceptual distortions experienced by those with dyslexia reflect a disturbance in the basic mechanisms supporting perceptual organization at the early stages of visual processing. Integration of information over extended areas of visual space can be measured psychophysically in a task that requires the detection of a path defined by aligned, spatially narrow-band elements on a dense field of otherwise similar elements that are randomly oriented and positioned. In the present study a contour integration task was used to investigate such perceptual organization in dyslexia. The detection of contours or paths composed of Gabor micropatterns was performed within a field of randomly oriented distracter elements in a 2-alternate forced choice (AFC) task. The stimuli were manipulated by ran...

ObjectiveMetabolic syndrome (MetS) is a cluster of metabolic abnormalities with insulin resistance (IR) as a major component. It has been recently questioned whether MetS and its related components are associated with functional and morphological alterations of the thyroid gland. The aim of our study is to examine thyroid volume and nodule prevalence in a case–control study of patients with MetS in a mild-to-moderate iodine-deficient area.DesignTwo hundred and seventy-eight patients with MetS were randomly matched for age, gender, and smoking habits with 261 subjects without MetS. Serum TSH, free tri-iodothyronine and thyroxine, and the level of IR, which was estimated by the homeostasis model assessment for IR, as well as other MetS parameters were evaluated. Thyroid ultrasonography was performed in all subjects. All subjects with thyroid nodules >1 cm were offered to undergo thyroid fine needle aspiration biopsy.ResultsTSH was significantly positively correlated with the presen...

All patients were assessed clinically at Agia Sofia Hospital in Athens. They belonged to three unrelated families from different regions of Greece. Their parents were healthy and nonconsanguineous. All pregnancies and perinatal periods were uneventful. Their ...

The effects of wood-ash fertilisation on tree stands, soil characteristics and ground vegetation were studied on a drained pine mire in Finland (64°51′N, 26°04′E, 62 m a.s.l.). The original site type was a treeless, mesotrophic Sphagnum papillosum fen. The site was drained in 1933 and the wood-ash fertilisation experiment was started in 1947. The treatments were: (i) unfertilised, (ii) wood-ash 8 t ha−1, and (iii) wood-ash 16 t ha−1.Drainage and ash application had radical and long-lasting consequences on the biological activity on the site and the vegetation compartments studied. The understorey vegetation had been profoundly affected by the ash with almost complete transformation of the species and other life forms. Even 50 years after the ash treatment the changes in vegetation/site type and the tree stand were clearly visible. On the unfertilised plot, the biomass of ground vegetation consisted mostly of mosses and dwarf shrubs, but on the ash-treated plots it consisted mostly of herbs and grasses typical of upland forests.Ash treatment had promoted stem volume growth of Scots pine (Pinus sylvestris L.) substantially and for a long time. The total wood production on the ash plots during 1947–1994 was 13 and 17 times over that of the control plot. Unfertilised pine trees suffered from P and K deficiency throughout the study period. The concentrations of some plant nutrients (P, K) decreased during the past years on Ash8. No nutrient shortage afflicting the tree stand was observed on Ash16 during the study period.Ash application has also led to increased concentration of nutrients in the peat. A sizeable proportion of the mineral nutrients applied were still in the 0–20 cm peat layer. On the ash-treated plots the amount of soil nitrogen (0–20 cm) was 18 and 29 times and the amount of soil phosphorus 9 and 13 times over the amount bound by the tree stand and the ground vegetation (Ash8 and Ash16, respectively). The stock of potassium was generally small in the surface peat—only 60–90% of the amount of potassium bound in the tree stand and the ground vegetation.It was concluded that wood-ash had powerfully influenced the biological processes in surface peat. The decomposition of cellulose was significantly accelerated by both ash treatments. Ash fertilisation also increased the emissions of CO2. The intensified decomposition rate in the litter, vegetation and peat explained to a large extent the accelerated growth of the Scots pine stands studied.

Survival and recovery of the seagrass Halophila ovalis (R.Br.) Hook during and after light deprivation was investigated to assist in the interpretation of recent losses of Halophila spp. in Queensland, Australia. Light deprivation experiments were conducted in outdoor aquaria and in situ at two water depths. Halophila ovalis plants were deprived of light for a maximum of 30 days, and recovery processes were investigated for up to 18 days following 15 days of light deprivation. Measurements of H. ovalis biomass, storage carbohydrate concentrations, chlorophyll a+b concentrations, stable carbon isotopes ratios (δ13C) and chlorophyll a fluorescence parameters (F0, Fm and Fv/Fm) were made during and at the end of the light deprivation and recovery periods. Biomass declined after 3–6 days in the dark and complete plant death occurred after 30 days. During the recovery period, biomass continued to decline for a short duration of time before stabilising. Sugar concentrations declined rapidly for the first 2 days of light deprivation before stabilising, then increased rapidly during the recovery period. Chlorophyll a+b concentrations were sensitive to very small differences in light availability: concentration decreased in total darkness, remained unchanged at 0.1% of surface irradiance and increased at 0.5% of surface irradiance. Photochemical efficiency of photosystem II (Fv/Fm) remained unchanged during the light deprivation and recovery periods. The lack of response in δ13C during light deprivation indicated the cessation of carbon fixation. Decreased sugar utilisation after 2 days of light deprivation indicated a reduction in respiration and growth. Starch concentrations did not change during light deprivation, suggesting the inhibition of starch utilisation by anaerobic conditions within the plant. Plant death after 30 days was notably faster than previously reported for other species of seagrass. The rapid die-off may be due to a shortage of available carbohydrates or due to a build-up of the phytotoxic end products of anaerobic respiration. Overall, H. ovalis has a very limited tolerance to light deprivation when compared to larger species of seagrass. Consequently, the persistence of this species in coastal marine environments may be dependent upon the occurrence and duration of transient light deprivation events.

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