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Conceived and designed the experiments: PC MP. Provided intellectual support and conceptual advice: MWH. A major challenge for the treatment of many central nervous system CNS disorders is the lack of convenient and effective methods for delivering biological agents to the brain. Mucopolysaccharidosis II Hunter syndrome is a rare inherited lysosomal storage disorder resulting from a deficiency of iduronatesulfatase I2S. I2S is a large, highly glycosylated enzyme. Intravenous administration is not likely to be an effective therapy for disease-related neurological outcomes that require enzyme access to the brain cells, in particular neurons and oligodendrocytes.
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Our house sat on a quiet street in a small town about 20 miles north of Birmingham, at that time our nearest neighbor was about a ten-minute walk away. Of course now the area is filled with subdivisions and yuppies, but at that time it was a peaceful quiet place to grow up. We had a low fence surrounding our backyard just to keep animals from tearing up the place but not high enough to keep anyone from getting in. Of course since our house was essentially surrounded by about 40 miles of forest there were no worries about burglars.
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