Mannosidosis: Separation and characterization of two acid α-mannosidase forms in mutant fibroblasts

Barbara K Burton, H. L. Nadler

Research output: Contribution to journalArticle

3 Scopus citations

Abstract

Two acidic forms of α-mannosidase activity, A and B, are separated and characterized in fibroblasts from controls and patients with mannosidosis. In normal cells, A and B differ in adsorption on DEAE-cellulose, electrophoretic mobility, stability at 70°C and resistance to freezing at -20°C. In 5 of 6 mutant cell lines, A and B both exhibit altered Km's for artificial substrates and decreased thermal stability. Zn2+ has no effect on A or B in mutant or normal cells. In contrast, Co2+ slightly inhibits B in normal cells but markedly enhances the activity of B in mutant cells. The mutation in mannosidosis clearly results in an alteration in the biochemical characteristics of both major acidic forms of α-mannosidase.

Original languageEnglish (US)
Pages (from-to)29-35
Number of pages7
JournalEnzyme
Volume23
Issue number1
DOIs
StatePublished - Jan 1 1978

ASJC Scopus subject areas

  • Biochemistry

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