Ann Pediatr Endocrinol Metab.  2018 Sep;23(3):131-135. 10.6065/apem.2018.23.3.131.

Therapeutic potential of alpha-1 antitrypsin in human disease

• Kim M
• Cai Q
• Oh Y

• KMID: 2422476
• DOI: http://doi.org/10.6065/apem.2018.23.3.131

Abstract

Alpha-1 antitrypsin (AAT), an alpha globulin glycoprotein, is a member of the serine protease inhibitor (serpin) superfamily. The clinical significance of AAT is highlighted by AAT deficiency. Genetic deficiency of AAT can present as several neutrophilic diseases associated with emphysema, liver cirrhosis, panniculitis, and systemic vasculitis. Recently, animal and human studies have shown that AAT can control inflammatory, immunological, and tissue-protective responses. In addition, AAT treatment can prevent overt hyperglycemia, increase insulin secretion, and reduce cytokine-mediated apoptosis of pancreatic β-cells in diabetes. These multifunctional roles of AAT draw attention to the glycoprotein's therapeutic potential for many inflammatory and autoimmune diseases beyond AAT deficiency. As underlying mechanisms, recent studies have suggested the importance of serine protease inhibitory activity of AAT in obesity-associated insulin resistance, chronic obstructive pulmonary disease, and cystic fibrosis. In this review, we explore the multiple functions of AAT, in particular, the anti-inflammatory and serine protease inhibitory functions, and AAT's therapeutic potential in a variety of human diseases through published literature.

Keyword

Alpha 1-antitrypsin; Therapeutic uses; Chronic obstructive pulmonary disease; Diabetes mellitus

MeSH Terms

alpha 1-Antitrypsin
Alpha-Globulins
Animals
Apoptosis
Autoimmune Diseases
Cystic Fibrosis
Diabetes Mellitus
Emphysema
Glycoproteins
Humans*
Hyperglycemia
Insulin
Insulin Resistance
Liver Cirrhosis
Neutrophils
Panniculitis
Pulmonary Disease, Chronic Obstructive
Serine Proteases
Systemic Vasculitis
Therapeutic Uses
Alpha-Globulins
Glycoproteins
Insulin
Serine Proteases
Therapeutic Uses
alpha 1-Antitrypsin

Reference

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