Comparative Study of Acid Maltase Deficiency: Biochemical Differences Between Infantile, Childhood, and Adult Types

Corrado Angelini, Andrew G. Engel

Research output: Contribution to journalArticle

80 Scopus citations

Abstract

Maltase (α-1,4-glucosidase) activity was determined at various pH values in tissues in infantile (heart, liver, skeletal muscle), childhood (heart, liver, skeletal muscle), and adult (liver, skeletal muscle, leukocytes) types of acid maltase deficiency (AMD) and in control tissues. In an infantile case of AMD, only traces of neutral maltase were present in heart, and this activity was significantly decreased in skeletal muscle and liver. In a childhood case of AMD, neutral maltase activity was decreased only in liver. No decreases of neutral maltase occurred in adult AMD tissues. An absolute decrease of leukocyte acid maltase was found in four and a relative decrease in one of five adults with AMD. A decrease in the pH 4.0/pH 6.5 ratio of leukocyte maltase activity may aid in the diagnosis of adult AMD.

Original languageEnglish (US)
Pages (from-to)344-349
Number of pages6
JournalArchives of neurology
Volume26
Issue number4
DOIs
StatePublished - Apr 1972

Keywords

  • Acidmaltase
  • glycogenosis
  • heart
  • leukocytes
  • liver
  • muscle
  • neutral maltase

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

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