| Literature DB >> 3278745 |
I Olsen1, D Abraham, I Shelton, G Bou-Gharios, H Muir, B Winchester.
Abstract
The activity of a lysosomal enzyme, alpha-D-mannosidase (EC 3.2.1.24), increased markedly in normal lymphocytes when they were cultured together with fibroblasts from a patient with an inherited deficiency of this enzyme. Cell-to-cell contact was obligatory for this increase in activity, which also required new protein synthesis. The enzyme induced in the co-cultured lymphocytes was a high molecular weight form of alpha-D-mannosidase that was not detected in lymphocytes cultured alone, which had only the low molecular weight mature enzyme. It was this precursor form alone that was directly transferred to the mannosidosis fibroblasts, where it was present initially in organelles of low density. When the culture period was extended the lymphocyte precursor enzyme was transported to the heavy lysosomes in the recipient cells, and correctly processed to the functionally effective mature enzyme.Entities:
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Year: 1988 PMID: 3278745 DOI: 10.1016/0167-4889(88)90022-5
Source DB: PubMed Journal: Biochim Biophys Acta ISSN: 0006-3002