Therapeutic potential of alpha-1 antitrypsin in human disease

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 cir...

Full description

Bibliographic Details
Main Authors: Minsun Kim, Qing Cai, Youngman Oh
Format: Article
Language:English
Published: Korean Society of Pediatric Endocrinology 2018-09-01
Series:Annals of Pediatric Endocrinology & Metabolism
Subjects:
Online Access:http://e-apem.org/upload/pdf/apem-2018-23-3-131.pdf
_version_ 1811272116913307648
author Minsun Kim
Qing Cai
Youngman Oh
author_facet Minsun Kim
Qing Cai
Youngman Oh
author_sort Minsun Kim
collection DOAJ
description 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.
first_indexed 2024-04-12T22:34:28Z
format Article
id doaj.art-de888bddd8474d5ebc559a58e04dbbe0
institution Directory Open Access Journal
issn 2287-1012
2287-1292
language English
last_indexed 2024-04-12T22:34:28Z
publishDate 2018-09-01
publisher Korean Society of Pediatric Endocrinology
record_format Article
series Annals of Pediatric Endocrinology & Metabolism
spelling doaj.art-de888bddd8474d5ebc559a58e04dbbe02022-12-22T03:13:54ZengKorean Society of Pediatric EndocrinologyAnnals of Pediatric Endocrinology & Metabolism2287-10122287-12922018-09-0123313113510.6065/apem.2018.23.3.131746Therapeutic potential of alpha-1 antitrypsin in human diseaseMinsun Kim0Qing Cai1Youngman Oh2 Department of Pediatrics, Chonbuk National University Medical School, Jeonju, Korea Department of Pathology, School of Medicine Medical College of Virginia Campus, Virginia Commonwealth University, Richmond, VA, USA Department of Pathology, School of Medicine Medical College of Virginia Campus, Virginia Commonwealth University, Richmond, VA, USAAlpha-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.http://e-apem.org/upload/pdf/apem-2018-23-3-131.pdfAlpha 1-antitrypsinTherapeutic usesChronic obstructive pulmonary diseaseDiabetes mellitus
spellingShingle Minsun Kim
Qing Cai
Youngman Oh
Therapeutic potential of alpha-1 antitrypsin in human disease
Annals of Pediatric Endocrinology & Metabolism
Alpha 1-antitrypsin
Therapeutic uses
Chronic obstructive pulmonary disease
Diabetes mellitus
title Therapeutic potential of alpha-1 antitrypsin in human disease
title_full Therapeutic potential of alpha-1 antitrypsin in human disease
title_fullStr Therapeutic potential of alpha-1 antitrypsin in human disease
title_full_unstemmed Therapeutic potential of alpha-1 antitrypsin in human disease
title_short Therapeutic potential of alpha-1 antitrypsin in human disease
title_sort therapeutic potential of alpha 1 antitrypsin in human disease
topic Alpha 1-antitrypsin
Therapeutic uses
Chronic obstructive pulmonary disease
Diabetes mellitus
url http://e-apem.org/upload/pdf/apem-2018-23-3-131.pdf
work_keys_str_mv AT minsunkim therapeuticpotentialofalpha1antitrypsininhumandisease
AT qingcai therapeuticpotentialofalpha1antitrypsininhumandisease
AT youngmanoh therapeuticpotentialofalpha1antitrypsininhumandisease