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2018 ; 11
(ä): 123-134
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The impact of alpha-1 antitrypsin augmentation therapy on neutrophil-driven
respiratory disease in deficient individuals
#MMPMID29618937
Dunlea DM
; Fee LT
; McEnery T
; McElvaney NG
; Reeves EP
J Inflamm Res
2018[]; 11
(ä): 123-134
PMID29618937
show ga
Alpha-1 antitrypsin (AAT) is the most abundant serine protease inhibitor
circulating in the blood. AAT deficiency (AATD) is an autosomal codominant
condition affecting an estimated 3.4 million individuals worldwide. The clinical
disease associated with AATD can present in a number of ways including COPD,
liver disease, panniculitis and antineutrophil cytoplasmic antibody vasculitis.
AATD is the only proven genetic risk factor for the development of COPD, and
deficient individuals who smoke are disposed to more aggressive disease.
Principally, AAT is a serine protease inhibitor; however, over the past number of
years, the assessment of AAT as simply an antiprotease has evolved, and it is now
recognized that AAT has significant anti-inflammatory properties affecting a wide
range of cells, including the circulating neutrophil.