alpha-1 antitrypsin

alpha-1 antitrypsin

A 54-kD glycoprotein that inhibits proteolytic enzymes (proteases), trypsin, chymotrypsin, elastases in lysosomes, plasmin, thrombin, collagenase and others; serum A1AT is a so-called acute-phase reactant.

Increased A1AT
Inflammation, liver injury, increased oestrogen, pregnancy, malignancy, corticosteroid therapy.
 
Decreased A1AT
Heterozygous A1AT deficiency, emphysema, prematurity, protein-losing disorders; by immunohistochemistry, A1AT stains atypical fibroxanthoma, granular cell tumour and papillary carcinoma of the thyroid.
 
Ref range
2–4 g/L
References in periodicals archive ?
Drug profiles discussed in this report include Alpha-1 Antitrypsin With rHuPH20, alpha-1 proteinase inhibitor (human), BB-3, EDO-66, MG-53, Monoclonal Antibody to Inhibit EMAP II for Emphysema, Recombinant Human Alpha-1 Antitrypsin and TRN-101.
American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency.
Recommendations for expensive treatments made for a genetic disorder called alpha-1 antitrypsin deficiency should be withdrawn because the drugs have no benefit, scientists said.
Washington, Dec 22 (ANI): People with single-gene mutations that cause them to have abnormally low levels of the protein alpha-1 antitrypsin are highly susceptible to emphysema-a progressive lung disease that causes severe shortness of breath.
The mission of the Alpha-1 Foundation is to provide the leadership and resources that will result in increased research, improved health, worldwide detection, and a cure for Alpha-1 Antitrypsin Deficiency.
Some 5,000 COPD patients will be tested to determine the prevalence of Alpha-1 Antitrypsin Deficiency (Alpha-1) in a study marking a major cooperative effort between the Alpha-1 Foundation and the American Association for Respiratory Care (AARC).
Articles in the literature on alpha-1 antitrypsin (AAT) deficiency have been interpreted as indicating that AAT deficiency is a rare disease that affects mainly Caucasians (whites) from northern Europe.
Currently extracted from human blood serum, alpha-1 antitrypsin is used to treat people who risk life-threatening emphysema because of an inherited deficiency of the enzyme.
Drug expands company's therapy offering to patients with alpha-1 antitrypsin deficiency
3, 2015 /PRNewswire/-- The Alpha-1 Foundation is marking Alpha-1 Awareness Month, designed to increase knowledge about Alpha-1 Antitrypsin Deficiency (Alpha-1).
Senior author Darrell Kotton, MD, an associate professor of medicine and pathology and co-director, Center for Regenerative Medicine at BUSM, said: "We applied this novel approach to achieve sustained in vivo expression of normal human alpha-1 antitrypsin (hAAT) protein at levels able to ameliorate emphysema in mice.

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