| Alpha-1
antitrypsin is a protein that is produced mostly in the liver. Its
primary function is to protect the lungs from neutrophil elastase.
Neutrophil elastase is an enzyme that normally serves a useful purpose
in the lung tissue – it digests damaged or aging cells and bacteria
to promote healing. However, if left unchecked, neutrophil elastase
will also attack healthy lung tissue.
Alpha-1 antitrypsin,
in sufficient amounts, will trap & destroy neutrophil elastase
before it has a chance to begin damaging the delicate (healthy)
lung tissue. Consequently, if an individual doesn’t have enough
alpha-1 antitrypsin, the enzyme goes unchecked and attacks the lung.
Most people
have two normal copies of the alpha-1 antitrypsin gene. People with
Alpha-1 (deficiency) may have one normal copy and one damaged copy,
or two damaged copies. Most Alphas with at least one normal gene
can produce enough alpha-1 antitrypsin to stay healthy.
People with
two damaged copies of the gene can’t produce enough alpha-1
antitrypsin, which can cause several conditions. They are often
diagnosed with emphysema as their primary disease. Other common
diagnoses include COPD, asthma, chronic bronchitis, and bronchiectasis.
Alphas are usually quite susceptible to lung infections. In the
Alpha-1 patient, any of these conditions can cause further damage
if they aren’t treated right away.
Alpha patients
can also develop cirrhosis of the liver. This scarring of healthy
liver tissue affects Alpha-1 infants, as well as about 15% of adult
Alphas. There is no cure for cirrhosis – except a transplant
– but if caught early, it can be managed as a chronic condition.
http://www.alpha1.org/
(this gives you links to 3 different sites)
http://www.nlm.nih.gov/medlineplus/ency/article/000120.htm
(gives a very detailed, illustrated explanation)
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