open access

Vol 24, No 1 (2018)
Review papers
Published online: 2018-04-12
Get Citation

The role of alpha-1-antitrypsin protein in the pathogenesis of abdominal aortic aneurysm

Agata Wanda Dżeljilji, Joanna Chorostowska-Wynimko, Dariusz Szewczyk, Wojciech Popiołek, Artur Kierach, Piotr Andziak
DOI: 10.5603/AA.2018.0006
·
Acta Angiologica 2018;24(1):30-33.

open access

Vol 24, No 1 (2018)
Review papers
Published online: 2018-04-12

Abstract

Alpha-1-antitrypsin is a potent antiprotease playing an important role in maintaining protease-antiprotease
balance. It protects the structures of extracellular matrix against destruction by proteolytic enzymes. Loss of
elasticity occurs when increased protease activity is accompanied by qualitative impairment or reduced concentrations
of antiproteases. Alpha-1-antitrypsin deficiency is a risk factor for obstructive lung disease, including
emphysema, liver and kidney disorders and, less often, follicular panniculitis, granulomatosis with polyangiitis
(previously Wegener’s granulomatosis). Literature also emphasises the role of AAT in the development of aortic
aneurysms, and results of biochemical studies support this theory. Aortic aneurysm is an important clinical
problem, unceasingly associated with high mortality. For this reason, it is exceptionally important to identify its
risk factors. Studies on the relationship between AAT and development of AAA (abdominal aortic aneurysm)
have been conducted since the 1990s. Due to the development in molecular diagnostic techniques, new reports
on the topic appeared over the last decade.

Abstract

Alpha-1-antitrypsin is a potent antiprotease playing an important role in maintaining protease-antiprotease
balance. It protects the structures of extracellular matrix against destruction by proteolytic enzymes. Loss of
elasticity occurs when increased protease activity is accompanied by qualitative impairment or reduced concentrations
of antiproteases. Alpha-1-antitrypsin deficiency is a risk factor for obstructive lung disease, including
emphysema, liver and kidney disorders and, less often, follicular panniculitis, granulomatosis with polyangiitis
(previously Wegener’s granulomatosis). Literature also emphasises the role of AAT in the development of aortic
aneurysms, and results of biochemical studies support this theory. Aortic aneurysm is an important clinical
problem, unceasingly associated with high mortality. For this reason, it is exceptionally important to identify its
risk factors. Studies on the relationship between AAT and development of AAA (abdominal aortic aneurysm)
have been conducted since the 1990s. Due to the development in molecular diagnostic techniques, new reports
on the topic appeared over the last decade.

Get Citation

Keywords

abdominal aortic aneurysm, alpha-1-antitrypsin

About this article
Title

The role of alpha-1-antitrypsin protein in the pathogenesis of abdominal aortic aneurysm

Journal

Acta Angiologica

Issue

Vol 24, No 1 (2018)

Pages

30-33

Published online

2018-04-12

DOI

10.5603/AA.2018.0006

Bibliographic record

Acta Angiologica 2018;24(1):30-33.

Keywords

abdominal aortic aneurysm
alpha-1-antitrypsin

Authors

Agata Wanda Dżeljilji
Joanna Chorostowska-Wynimko
Dariusz Szewczyk
Wojciech Popiołek
Artur Kierach
Piotr Andziak

References (32)
  1. Brown SL, Lundgren CH, Nordt T, et al. Stimulation of migration of human aortic smooth muscle cells by vitronectin: implications for atherosclerosis. Cardiovascular Research. 1994; 28(12): 1815–1820.
  2. Wassef M, Baxter BT, Chisholm RL, et al. Pathogenesis of abdominal aortic aneurysms: a multidisciplinary research program supported by the National Heart, Lung, and Blood Institute. J Vasc Surg. 2001; 34(4): 730–738.
  3. Busuttil RW, Rinderbriecht H, Flesher A, et al. Elastase activity: the role of elastase in aortic aneurysm formation. J Surg Res. 1982; 32(3): 214–217.
  4. Cannon DJ, Read RC. Blood elastolytic activity in patients with aortic aneurysm. Ann Thorac Surg. 1982; 34(1): 10–15.
  5. Całkosiński I, Dobrzyński M, Całkosińska M, et al. Bronowicka- Szydełko A, Dzierzba K, Ceremuga I, Damian D. Charakterystyka odczynu zapalnego. Postępy Hig Med Dosw. 2009; 63: 395–408.
  6. Roszkowska- Ja, Worowska A, Gacko M, et al. Proteazy granulocytów obojętnochłonnych. Post Hig Med Dośw. 2002; 56: 73–92.
  7. Chorostowska- Wy, Popławska B, Janciauskiene S. Alfa-1 antytrypsyna: rola w fizjologii i patologii człowieka. Int Rev Allergol Clin Immunol Family Med. 2012; 18: 22–28.
  8. Popławska B, Janciauskiene S. Chorostowska- Wynimko J. Genetyczne warianty alfa-1 antytrypsyny: klasyfikacja i znaczenie kliniczne. Pneumonol Alergol Pol. . 2013; 81(1): 45–54.
  9. Chorostowska-Wynimko J, Niżankowska-Mogilnicka E, Bakuła A, et al. Zasady postępowania diagnostycznego i opieki nad chorými z wrodzonym niedoborem alfa-1 antytrypsyny. Pneumonol Alergol Pol. 2010; 78(3): 348–355.
  10. Köhnlein T, Janciauskiene S, Welte T. Diagnostic delay and clinical modifiers in alpha-1 antitrypsin deficiency. Ther Adv Respir Dis. 2010; 4(5): 279–287.
  11. Opolska B. Badania nad częstością występowania fenotypów układu PI w populacji Polski południowej. Przegl Lek. 1974; 31: 851–854.
  12. Kaczor MP, Sanak M, Libura-Twardowska M, et al. The prevalence of alpha(1)-antitrypsin deficiency in a representative population sample from Poland. Respir Med. 2007; 101(12): 2520–2525.
  13. Titenko-Holland NV, Kowalska A. Alpha-1-antitrypsin (PI) subtypes in Russians and Poles. Hum Hered. 1992; 42(6): 384–386.
  14. Kowalska A, Rujner J. Polimorfizm lokus PI (alpha-1 antitrypsin) u mieszkańców województwa poznańskiego. Pol Tyg Lek. 1994; 49: 195–197.
  15. Kowalska A, Rujner J, Titenko-Holland NV, et al. Alpha-1-antitrypsin subtypes in Polish newborns. Hum Hered. 1995; 45(6): 351–354.
  16. Chorostowska- Wy, Struniawski R, Popławska B. Borszewska- Kornacka M. Ocena częstości występowania głównych alleli deficytowych genu alfa-1 antytrypsyny w populacji województwa mazowieckiego- wstępne wyniki badania przesiewowego noworodków. Pneumol Alergol Pol 2012;80. ; 5: 450–453.
  17. Stoller JK, Sandhaus RA, Turino G, et al. Delay in diagnosis of alpha1-antitrypsin deficiency: a continuing problem. Chest. 2005; 128(4): 1989–1994.
  18. Chorostowska-Wynimko J, Struniawski R, Sliwinski P, et al. The National Alpha-1 Antitrypsin Deficiency Registry in Poland. COPD: Journal of Chronic Obstructive Pulmonary Disease. 2015; 12(sup1): 22–26.
  19. Tilson MD, Seashore MR. Fifty families with abdominal aortic aneurysms in two or more first-order relatives. Am J Surg. 1984; 147(4): 551–553.
  20. Johansen K, Koepsell T. Familial tendency for abdominal aortic aneurysms. JAMA. 1986; 256(14): 1934–1936.
  21. Cohen JR, Mandell C, Margolis I, et al. Altered aortic protease and antiprotease activity in patients with ruptured abdominal aortic aneurysms. Surg Gynecol Obstet. 1987; 164(4): 355–358.
  22. Cohen JR, Mandell C, Chang JB, et al. Elastin metabolism of the infrarenal aorta. J Vasc Surg. 1988; 7(2): 210–214.
  23. Cohen JR, Sarfati I, Ratner L, et al. Alpha 1-antitrypsin phenotypes in patients with abdominal aortic aneurysms. J Surg Res. 1990; 49(4): 319–321.
  24. Schardey HM, Hernandez-Richter T, Klueppelberg U, et al. Alleles of the alpha-1-antitrypsin phenotype in patients with aortic aneurysms. J Cardiovasc Surg (Torino). 1998; 39(5): 535–539.
  25. Pini L, Bonardelli S, Ferrarotti I, et al. Alpha-1 antitrypsin deficiency and abdominal aortic aneurysms: does this association really exist? Am J Respir Crit Care Med . 2015; 191: A2745.
  26. Elzouki AN, Rydén Ahlgren A, Länne T, et al. Abdominal aortic aneurysms and alpha 1-antitrypsin deficiency. J Intern Med. 1994; 236(5): 587–591.
  27. Ramsbottom D, Fitzgerald P, Grace PA, et al. Biochemical and molecular genetic studies of abdominal aortic aneurysm in an Irish population. Eur J Vasc Surg. 1994; 8(6): 716–722.
  28. Molacek J, Mares J, Treska V, et al. Proteomic analysis of the abdominal aortic aneurysm wall. Surg Today. 2014; 44(1): 142–151.
  29. Boomsma DI, Orbeleke JF, Martin NG, et al. Alpha-1-antitrypsin and blood pressure. Lancet. 1991; 337(8756): 1547.
  30. Elzouki AN, Rydén Ahlgren A, Länne T, et al. Is there a relationship between abdominal aortic aneurysms and alpha1-antitrypsin deficiency (PiZ)? Eur J Vasc Endovasc Surg. 1999; 17(2): 149–154.
  31. Ahlgren AR, Piitulainen E, Sonesson B, et al. Changes in aortic wall stiffness in men with alpha 1-antitrypsin deficiency. Eur J Vasc Endovasc Surg. 1997; 14(4): 252–257.
  32. American Thoracic Society, European Respiratory Society. American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med. 2003; 168(7): 818–900.

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

By "Via Medica sp. z o.o." sp.k., ul. Świętokrzyska 73, 80–180 Gdańsk

tel.:+48 58 320 94 94, faks:+48 58 320 94 60, e-mail: viamedica@viamedica.pl