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