Disaccharidase activities in dyspeptic children: Biochemical and molecular investigations of maltase-glucoamylase activity

Wikrorn Karnsakul, Ursula Luginbuehl, Dagmar Hahn, Erwin Sterchi, Stephen Avery, Partha Sen, Dallas Swallow, Buford Nichols*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

36 Scopus citations

Abstract

Background: Maltase-glucoamylase enzyme plays an important role in starch digestion. Glucoamylase deficiency is reported to cause chronic diarrhea in infants, but its role in dyspeptic children is unknown. Methods: Glucoamylase and other disaccharidase specific activities were assayed from duodenal biopsy specimens in 44 children aged 0.5-18 years (mean, 10 ± 5 years) undergoing endoscopy to evaluate dyspeptic symptoms. All subjects had normal duodenal histology. Intestinal organ culture was used to evaluate synthesis and processing of maltase-glucoamylase. Sequencing of the maltase-glucoamylase coding region was performed in subjects with low activity or variation of isoform in organ culture. Results: Twenty-two of the dyspeptic children had one or more disaccharidases with low specific activity. Twelve subjects (28%) had low activity of glucoamylase. Eight subjects had low activities of glucoamylase, sucrase, and lactase. Low glucoamylase activity was not correlated with the isoform phenotype of maltase-glucoamylase as described by metabolic labeling and sodium dodecyl sulfate electrophoresis. Novel nucleotide changes were not detected in one subject with low glucoamylase activity or in two subjects with variant isoforms maltase-glucoamylase peptides. Conclusion: Twelve of 44 dyspeptic children had low specific activity of duodenal maltase-glucoamylase. Eight of these children had low specific activity of all measured disaccharidases.

Original languageEnglish (US)
Pages (from-to)551-556
Number of pages6
JournalJournal of pediatric gastroenterology and nutrition
Volume35
Issue number4
DOIs
StatePublished - Oct 2 2002
Externally publishedYes

Keywords

  • Lactase-phlorizin hydrolase
  • Maltase-glucoamylase
  • Multiple disaccharidase deficiency
  • Sucrase-isomaltase

ASJC Scopus subject areas

  • Gastroenterology
  • Pediatrics, Perinatology, and Child Health

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