Monoclonal antibody against polyglucosan isolated from the myocardium of a patient with Lafora disease.

ABSTRACT

Polyglucosan was extracted from the myocardium of a patient with Lafora disease. Gas chromatographic analysis of the isolated polyglucosan revealed glucose as the only sugar component. Monoclonal antibodies (MAb) were raised against the isolated polyglucosan. With the MAb, immunocytochemistry showed positive staining of Lafora bodies, corpora amylacea, basophilic degeneration of the cardiac muscle cells, and inclusion bodies within cardiac muscle cells and the hepatocytes of patients with Lafora disease and type IV glycogenosis (Andersen's disease). These findings indicate that cells of the latter group share antigenicity with Lafora bodies.