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Related Experiment Video

Updated: May 13, 2026

Detecting Glycogen in Peripheral Blood Mononuclear Cells with Periodic Acid Schiff Staining

Detecting Glycogen in Peripheral Blood Mononuclear Cells with Periodic Acid Schiff Staining

Published on: December 23, 2014

Cardiac Pathology in Glycogen Storage Disease Type III.

S L Austin¹, A D Proia, M J Spencer-Manzon

¹Departments of Pediatrics, Duke University Medical Center, GSRB1, 595 South La Salle Street, DUMC 103857, Durham, NC, 27710, USA, stephanie.austin@duke.edu.

|February 23, 2013

Summary

Glycogen accumulation in heart muscle and vessels is common in Glycogen Storage Disease (GSD) III. This can lead to heart failure and arrhythmias in affected individuals.

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Last Updated: May 13, 2026

Detecting Glycogen in Peripheral Blood Mononuclear Cells with Periodic Acid Schiff Staining

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Published on: December 23, 2014

Biochemical Titration of Glycogen In vitro

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Published on: November 24, 2013

The Extraction of Liver Glycogen Molecules for Glycogen Structure Determination

The Extraction of Liver Glycogen Molecules for Glycogen Structure Determination

Published on: February 8, 2022

Area of Science:

Cardiology
Genetics
Pathology

Background:

Glycogen Storage Disease (GSD) III is a rare genetic disorder affecting glycogen metabolism.
Cardiac involvement is a known complication of GSD III, but its specific manifestations require further elucidation.

Purpose of the Study:

To investigate the distribution and clinical impact of glycogen accumulation on heart structure and function in individuals with GSD III.

Main Methods:

Examination of cardiac tissue and clinical records from three GSD IIIa patients.
Analysis included macro- and microscopic assessment of cardiac structures.

Main Results:

Observed cardiac fibrosis, myocyte vacuolation, and glycogen accumulation in the AV node.
Significant glycogen deposition in intramyocardial arteries led to hyperplasia and thickened vascular walls.

Conclusions:

Findings demonstrate diffuse cardiac involvement in GSD III patients.
Potential for serious arrhythmias and symptomatic heart failure necessitates careful patient management.