Alpha1-antitrypsin genetic polymorphism in ANCA-positive systemic vasculitis. Alpha1-antitrypsin (α1-AT) is the major inhibitor of proteinase 3 (PR3), the main target antigen of antineutrophil cytoplasm antibodies (ANCA) in Wegener's granulomatosis. α1-AT is encoded by a polymorphic gene, with over 75 alleles, defining severely, medium and non-deficient protease inhibitor (PI) phenotypes. We describe the association of severely and medium deficient PI phenotypes with anti-PR3 positive systemic vasculitis, and postulate a pathogenetic role for α1-AT deficiency and the occurrence of ANCA, with specificity for PR3 in a subgroup of patients with Wegener's granulomatosis
In the sera of patients with Wegener's granulomatosis (WG), C-ANCA can be detected that are directed...
The proteinase 3 (PR3)-positive anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculiti...
Antiproteinase 3- and antimyeloperoxidase-associated vasculitis. Wegener's granulomatosis, microscop...
Proteinase 3 gene polymorphisms and Wegener's granulomatosis.BackgroundWegener's granulomatosis (WG)...
BACKGROUND Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a severe condition en...
Inhibition of proteinase 3 by ANCA and its correlation with disease activity in Wegener's granulomat...
The genetics of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a complex...
We have previously demonstrated that a strong correlation exists between systemic vasculitis with pr...
Background Small studies have linked alpha 1 antitrypsin (alpha 1AT) deficiency to patients with ant...
α1-Antitrypsin (α1-AT) deficiency is a common genetic disorder characterized by low serum α1-AT leve...
Introduction: Anti-proteinase 3 and anti-myeloperoxydase vasculitis are associated with an overrepre...
Abstract. Objectives. To ascertain whether a relationship exists between the PiZ alpha1‐antitrypsin ...
Alpha1-antitrypsin (D,1AT) is the most abundant circulating protease inhibitor (Pi) in human plasma....
The deficiency of alpha-1 protease inhibitor, or alpha-1-antitrypsin (A1AT), predisposes to chronic ...
Antineutrophil cytoplasmic antibodies (ANCA) have been described as sensitive and specific markers f...
In the sera of patients with Wegener's granulomatosis (WG), C-ANCA can be detected that are directed...
The proteinase 3 (PR3)-positive anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculiti...
Antiproteinase 3- and antimyeloperoxidase-associated vasculitis. Wegener's granulomatosis, microscop...
Proteinase 3 gene polymorphisms and Wegener's granulomatosis.BackgroundWegener's granulomatosis (WG)...
BACKGROUND Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a severe condition en...
Inhibition of proteinase 3 by ANCA and its correlation with disease activity in Wegener's granulomat...
The genetics of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a complex...
We have previously demonstrated that a strong correlation exists between systemic vasculitis with pr...
Background Small studies have linked alpha 1 antitrypsin (alpha 1AT) deficiency to patients with ant...
α1-Antitrypsin (α1-AT) deficiency is a common genetic disorder characterized by low serum α1-AT leve...
Introduction: Anti-proteinase 3 and anti-myeloperoxydase vasculitis are associated with an overrepre...
Abstract. Objectives. To ascertain whether a relationship exists between the PiZ alpha1‐antitrypsin ...
Alpha1-antitrypsin (D,1AT) is the most abundant circulating protease inhibitor (Pi) in human plasma....
The deficiency of alpha-1 protease inhibitor, or alpha-1-antitrypsin (A1AT), predisposes to chronic ...
Antineutrophil cytoplasmic antibodies (ANCA) have been described as sensitive and specific markers f...
In the sera of patients with Wegener's granulomatosis (WG), C-ANCA can be detected that are directed...
The proteinase 3 (PR3)-positive anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculiti...
Antiproteinase 3- and antimyeloperoxidase-associated vasculitis. Wegener's granulomatosis, microscop...