Alpha-1 Antitrypsin Deficiency, also known as Alpha-1, is an inherited disorder that causes significant reduction in the naturally occurring protein alpha1 antitrypsin (AAT). Scientists also call this protein Alpha-1 Proteinase Inhibitor (A 1 Pi) because it inhibits not only trypsin but also other enzymes called proteinases. It is believed that Alpha-1 affects as many as 100,000 people in the United States and similar numbers in Europe. Alpha-1 is most common among Caucasians of Northern European and Iberian descent. It is the most common cause of genetic liver disease in children and genetic emphysema in adults.
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