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Altered serum α-D-mannosidase activity in mucolipidosis II and mucolipidosis III

Authors
Journal
Biochemical and Biophysical Research Communications
0006-291X
Publisher
Elsevier
Publication Date
Volume
81
Issue
3
Identifiers
DOI: 10.1016/0006-291x(78)91416-x
Disciplines
  • Chemistry
  • Medicine

Abstract

Abstract Normal human serum contains at least three forms of α-D-mannosidase: an acidic form which has a pH optimum of 4.25, is inhibited by Co 2+ and is thermostable; an intermediate form, which has a pH optimum of 5.6–5.7, is stimulated by Co 2+ and is heat labile at 50°C; and a neutral form with a pH optimum of 6.0–6.5. In Mucolipidosis II and III sera, the acidic α-mannosidase activity persists while the intermediate activity is absent or altered. Heating the serum does not affect the pH activity curve, the electrofocusing profile or the response to Co 2+ of α-mannosidase. During heat inactivation at 55°, 90–100% of the pH 5.6 α-mannosidase activity is lost in normal sera while less than 40% is lost from ML sera. The effect on sera from ML obligate heterozygotes is intermediate. The absent or altered intermediate mannosidase may be responsible for aberrant biochemical properties reported for other glycosidases in Mucolipidosis II and Mucolipidosis III.

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