Question

α - 1 antitrypsin is

• A. an antacid
• B. an enzyme
• C. used to treat emphysema
• D. used to treat arthritis

Answer 1

The Correct Option is C

Let's analyze the nature and function of α-1 antitrypsin (AAT): 

• What it is: α-1 antitrypsin is a protein, specifically a serine protease inhibitor (serpin). It is naturally produced mainly by the liver and circulates in the blood.
• What it does: Its primary role is to protect the body's tissues from being damaged by enzymes released from immune cells, particularly neutrophil elastase. Neutrophil elastase is an enzyme released by neutrophils (a type of white blood cell) during inflammation, and its main function is to break down harmful bacteria. However, if unregulated, it can also break down elastin, a key component of lung tissue elasticity. AAT inhibits neutrophil elastase, thus protecting the lungs.
• Deficiency: Genetic deficiency of α-1 antitrypsin (AATD) leads to insufficient protection of the lungs from neutrophil elastase. This results in progressive damage to the lung alveoli, leading to conditions like emphysema (a type of chronic obstructive pulmonary disease, COPD). It can also cause liver disease due to the accumulation of abnormal AAT protein in liver cells.
• Therapeutic Use: For individuals with severe AATD and related lung disease, augmentation therapy using purified α-1 antitrypsin protein (obtained from human plasma or produced via recombinant DNA technology) is administered intravenously. This therapy aims to increase the levels of AAT in the lungs, providing protection against elastase and slowing the progression of emphysema.

Now let's evaluate the options:

• an antacid: Incorrect. Antacids neutralize stomach acid.
• an enzyme: Incorrect. It is a protease inhibitor, which blocks the activity of enzymes.
• used to treat emphysema: Correct. Specifically, it is used as augmentation therapy for emphysema caused by α-1 antitrypsin deficiency.
• used to treat arthritis: Incorrect. While inflammation is part of arthritis, AAT therapy is specifically targeted for AATD-related lung disease.

Therefore, α-1 antitrypsin is used to treat emphysema (associated with its deficiency).

Answer 2

α-1 antitrypsin is a protein produced in the liver and released into the bloodstream. It plays a crucial role in protecting the lungs by inhibiting an enzyme called neutrophil elastase, which can damage alveoli (air sacs) in the lungs if not properly controlled.

In α-1 antitrypsin deficiency, this protective protein is lacking or defective, leading to unchecked activity of neutrophil elastase, which damages lung tissue and can cause a condition called emphysema, a chronic obstructive pulmonary disease (COPD).

Therapeutic use:
α-1 antitrypsin is used as a treatment for emphysema caused by its deficiency. This therapy involves intravenous infusions of purified α-1 antitrypsin to restore its levels in the blood and protect the lungs.

Correct Answer: used to treat emphysema