Glycogen phosphorylase isoenzyme BB (GPBB) is a key enzyme of glycogenolysis. Its degree of association with the sarcoplasmatic reticulum glycogenolysis complex depends essentially on the metabolic state of the myocardium. With the onset of tissue hypoxia, when glycogen is broken down, GPBB is converted from a structurally bound into a cytoplasmatic form. Considerable amounts of GPBB are only found in human heart and brain. In the first clinical studies GPBB was the most sensitive marker for the diagnosis of acute myocardial infarction within 4 h of chest pain onset. GPBB also increases early in patients with unstable angina and reversible ST-T alterations in the resting electrocardiogram at hospital admission, which could be useful for risk stratification. GPBB is sensitive for the detection of perioperative ischaemic myocardial damage and infarction in patients undergoing coronary artery bypass grafting. The diagnostic specificity of GPBB in non-traumatic chest pain patients was comparable to creatine kinase MB. These results indicate that GPBB is a sensitive marker for ischaemic myocardial damage.