Intracellular retention of lysosomal enzymes in cystic fibrosis

Multiple leakage of lysosomal enzymes from cystic fibrosis fibroblasts into the extracellular space has been suggested to occur but was not observed in these experiments. Both extracellular and intracellular levels of enzyme activity were within the normal range. The release of lysosomal enzymes from individual cell lines was seen to be affected both by intrinsic properties of each cell line and by many external factors.