Enzyme-cell membrane recognition (what we can learn from rare genetic lysosomal disorders)

U. N. Wiesmann, S. Di Donato

Research output: Contribution to journalArticlepeer-review


Evidence is presented that the uptake of lysosomal enzymes into normal and into enzyme deficient fibroblasts is a very complex process, governed by the structure of the lysosomal enzyme and the receptors of the cell membrane. The number of receptors in the cell membrane may not be the same for all cells in the organism. Lysosomal enzymes that were infused into patients rapidly disappeared from the circulation and were almost instantly accumulated in the cells of the reticuloendothelial system such as the 'Kupffer' cells in the liver and the reticulum cells in the spleen. Such observations may be better understood in the light of the recent findings that those cells are the ones which have the appropriate binding sites for the lysosomal enzymes at their cell surface, whereas other cells may not.

Original languageEnglish
Pages (from-to)7-14
Number of pages8
JournalBiochemistry and Experimental Biology
Issue number1
Publication statusPublished - 1977

ASJC Scopus subject areas

  • Medicine(all)


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