2.Futerman2004%20pp554 65

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2.Futerman2004%20pp554 65

  1. 1. REVIEWS THE CELL BIOLOGY OF LYSOSOMAL STORAGE DISORDERS Anthony H. Futerman* and Gerrit van Meer‡ Lysosomal storage disorders, of which more than 40 are known, are caused by the defective activity of lysosomal proteins, which results in the intra-lysosomal accumulation of undegraded metabolites. Despite years of study of the genetic and molecular bases of lysosomal storage disorders, little is known about the events that lead from this intra-lysosomal accumulation to pathology. Here, we summarize the biochemistry of lysosomal storage disorders. We then discu

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