Metabolic Studies of Glycosphingolipid Accumulation in Mucopolysaccharidosis IIID

Severe neurological deficits and mental retardation are frequently associated with disrupted ganglioside metabolism in a variety of gangliosidoses and lysosomal storage disorders. Accumulation of glycosphingolipids (GSLs) in the central nervous system (CNS) of humans and animals affected with severa...

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Veröffentlicht in:	Molecular genetics and metabolism 2001-03, Vol.72 (3), p.239-247
Hauptverfasser:	Liour, Sean S., Jones, Margaret Z., Suzuki, Minoru, Bieberich, Erhard, Yu, Robert K.
Format:	Artikel
Sprache:	eng
Schlagworte:	Age Factors Animals Brain - metabolism glycosphingolipids Glycosphingolipids - metabolism Goats lysosomal storage Lysosomes - metabolism Mice mucopolysaccharidosis Mucopolysaccharidosis III - genetics Mucopolysaccharidosis III - metabolism N-Acetylgalactosaminyltransferases - metabolism Neuraminidase - metabolism neurological disorder Polypeptide N-acetylgalactosaminyltransferase Suramin - pharmacology Tumor Cells, Cultured
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