Change of isoforms' spectra of alpha-L-fucosidase from human skin fibroblasts in intracellular storage of nonhydrolyzable substances

Biochim Biophys Acta. 1995 Jan 25;1270(1):7-11. doi: 10.1016/0925-4439(94)00062-u.

Abstract

The effect of exogenous and endogenous products storage in lysosomes on the activity and multiple forms of alpha-L-fucosidase from human skin fibroblasts was investigated. It was shown that sucrose load, modelling intralysosomal accumulation of nonhydrolyzable products, causes certain changes in secretion level of alpha-L-fucosidase and multiple forms' spectra of the intracellular and secreted enzymes. These changes were different for the enzyme from embryonal and postnatal normal fibroblasts. Some changes of secreted alpha-L-fucosidase isoforms' spectra were found in fibroblasts from a patient with Fabry's disease, characterized by the intralysosomal storage of di- and trihexosylceramides. The alterations of isoforms' profiles in Fabry fibroblasts at the early and late accumulation stages were similar to those in sucrose-loaded embryonal and postnatal fibroblasts, respectively. It is proposed that intralysosomal accumulation of nonhydrolyzable compounds influences the alpha-L-fucosidase posttranslational processing.

MeSH terms

  • Cells, Cultured
  • Fibroblasts / enzymology
  • Fibroblasts / pathology
  • Humans
  • Isoelectric Focusing
  • Lysosomal Storage Diseases / enzymology
  • Lysosomal Storage Diseases / pathology
  • Skin / embryology
  • Skin / enzymology*
  • alpha-L-Fucosidase / chemistry*

Substances

  • alpha-L-Fucosidase