Alpha-1 Antitrypsin Deficiency

Reviewed by Robert A. Sandhaus, MD, PhD, FCCP

Alpha-1 antitrypsin is a major protein in the blood that it is produced mainly in liver cells. When you don't have enough alpha-1 antitrypsin, you might have Alpha-1 Antitrypsin Deficiency, sometimes called Alpha-1. Alpha-1 can affect two major organs in the body - the lungs and the liver. The reason is because the defective protein that characterizes Alpha-1 is important to both of these vital organs.

  • When Alpha-1 affects the lungs, it can cause COPD and is called Alpha-1 Deficiency or Alpha-1 emphysema.

  • When Alpha-1 affects the liver, it is called Alpha-1 liver disease.

  • There may also be an association between Alpha-1 and atypical TB infections in the lung, also called Non-Tuberculous Mycobacteria.

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