Tab. 6. Mechanisms of intracellular accumulation of substances (Intracellular Accumulations or parenchymal dystrophies) 1. Normal cell 13 Protein mutation 0 Complex Soluble substrate products Enzyme (1) Abnormal metabolism. 2 Defect in protein folding, transport -X- 3 Lack of enzyme Fatty liver 4 Ingestion of indigestible materials CH Accumulation of abnormal proteins Complex substrate Lysosomal storage disease accumulation of endogenous materials Accumulation of exogenous materials 2. 3. 4.

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