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Ann Am Thorac Soc. 2016 Aug;13 Suppl 4:S336-40. doi: 10.1513/AnnalsATS.201509-574KV.

Biomarkers in Alpha-1 Antitrypsin Deficiency Chronic Obstructive Pulmonary Disease.

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1 Mt. Sinai-St. Luke's-Roosevelt Hospital, New York, New York.
2 Mt. Sinai Icahn School of Medicine, New York, New York; and.
3 St. John's University College of Pharmacy and Health Sciences, New York, New York.


Biomarkers of pathogenesis in chronic obstructive pulmonary disease (COPD) can significantly accelerate drug development. In COPD related to alpha-1 antitrypsin deficiency, the role of neutrophil elastase and its inhibition by alpha-1 antitrypsin protein focused interest on elastin degradation and the development of pulmonary emphysema. Amino acids desmosine and isodesmosine are unique cross-links in mature elastin fibers and can serve as biomarkers of elastin degradation when measured in body fluids. This review gives a perspective on what has been learned by the earliest measurements of desmosine and isodesmosine followed by later studies using methods of increased sensitivity and specificity and the meaning for developing new therapies. Also included are brief statements on the biomarkers fibrinogen, CC-16, and Aa-Val-360 in COPD.


alpha-1 antitrypsin deficiency; biomarkers; chronic obstructive pulmonary disease; emphysema

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