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Metabolic Studies of Glycosphingolipid Accumulation in Mucopolysaccharidosis IIID

✍ Scribed by Sean S. Liour; Margaret Z. Jones; Minoru Suzuki; Erhard Bieberich; Robert K. Yu

Book ID
115639815
Publisher
Elsevier Science
Year
2001
Tongue
English
Weight
142 KB
Volume
72
Category
Article
ISSN
1096-7192

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In the normal C57BL/6J male mouse a specific subset of the kidney glycosphingolipids which is associated with multilamellar bodies of lysosomal origin and represents about 10% of the total kidney glycolipids, is excreted into the urine each day. This excretion is blocked and glycosphingolipids accum