Storage diseases are the consequence of inherited enzyme deficiencies that lead to the accumulation of substances that are incompletely catabolised in the cells of the mononuclear phagocyte system in organs such as the liver, spleen and bone marrow. Many of these products are derived from lipids of the cell membrane.

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Disorders of the Mononuclear Phagocyte System

  • Reiner Bartl,
  • Christoph Bartl

摘要

Storage diseases are the consequence of inherited enzyme deficiencies that lead to the accumulation of substances that are incompletely catabolised in the cells of the mononuclear phagocyte system in organs such as the liver, spleen and bone marrow. Many of these products are derived from lipids of the cell membrane.