A 28-year-old man was found to have cirrhosis of the liver and pulmonary emphysema. The liver cells contained globular inclusions in their cytoplasm, which by electron microscopy are shown to be located inside the

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The clinical history (i.e., the concurrence of cirrhosis and pulmonary emphysema) suggests that this young man has a₁-antitrypsin (AAT) deficiency. The cytoplasmic globules in the liver cells represent misfolded AAT, which cannot be excreted from the liver cells and remains inside the liver cells in the dilated cisterns of the rough endoplasmic reticulum.

A 28-Year-Old Man Was Found to Have Cirrhosis of the Liver