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Mucopolysaccharidosis type VI, Maroteaux-Lamy syndrome is a lysosomal storage disorder with progressive, multisystem involvement caused by deficiency of the lysosomal enzyme N-acetylgalactosamine-4-sulfatase leading to accumulation of the glycosaminoglycan, keratan sulfate. Enzyme replacement therapy (ERT) has been shown to clinically benefit affected(More)
2 patients with fast and 4 with medium varieties of Gaucher's syndrome were treated by displacement bone marrow transplantation to install a donor enzyme factory for life. Immunoprophylaxis was given to prevent host impairment of normal enzymes. 5 patients showed rapid and remarkable improvement and were leading fully active lives at 310-1207 days(More)
HABITAT MAPPING Habitat maps were ground-truthed using a handheld GPS enabled PC with Arc Pad software (accuracy ± 4m) using the habitat categories (Table S5). Hedgerows and tree lines were mapped using Google maps aerial images (Google 2013) and area was calculated by multiplying the length by a mean width of 1.93 m (data from 14 hedges in Berkshire and(More)
Anthropogenic changes to the temperature regimes of rivers, whether through thermal pollution, removal of shade, or climate change, could affect community stability and cause phenological changes in aquatic species. This study examines the impact of a thermal discharge from a power station on the diversity and composition of the aquatic macroinvertebrate(More)