Wegener's autoantigen decoded

Sir-Autoantibodies directed against a unique antigen in cytoplasmic granules of human neutrophils ACPA are a diseasespecific marker for Wegener's granulomatosis, a systemic vasculitis complicated by myelo-monocyte proliferation1. Because disease activity and autoantibody titre are closely corre...

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Bibliographic Details
Published in:Nature (London) 1990-08, Vol.346 (6284), p.520-520
Main Authors: JENNE, D. E, TSCHOPP, J, LÜDEMANN, J, UTECHT, B, GROSS, W. L
Format: Article
Language:English
Subjects:
Amino Acid Sequence
Antigens
Autoantibodies
Autoantigens - genetics
Autocrine signalling
Base Sequence
Elastase
Granulomatosis
Granulomatosis with Polyangiitis - genetics
Granulomatosis with Polyangiitis - immunology
Growth factors
Humans
Leukocytes (granulocytic)
Leukocytes (neutrophilic)
Leukocytosis
Lymphocytes T
Mast cells
Molecular Sequence Data
Monocytes
Neutrophils
Peptides
Proteinase
Proteinase 3
Sequence Homology, Nucleic Acid
Serine
Systemic vasculitis
Wegener's granulomatosis
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Summary:Sir-Autoantibodies directed against a unique antigen in cytoplasmic granules of human neutrophils ACPA are a diseasespecific marker for Wegener's granulomatosis, a systemic vasculitis complicated by myelo-monocyte proliferation1. Because disease activity and autoantibody titre are closely correlated, autoantibodies are regarded as an important pathogenetic factor in this disease. Human neutrophil elastase and proteinase 3/myeloblastin are closely related with neutral serine proteases of other haematopoietic cell types, in particular with serine proteases of mast cells and with a family of lymphocyte granzymes1' whose functions are not known. [...]there is evidence that murine granzyme A can act as an autocrine growth factor with mitogenic activity for X-ray-induced T-cell lymphomas7. Because most autoantibodies against proteinase 3/myeloblastin interfere with its enzymatic function (data not shown), excessive neutrophilic leukocytosis seen in fulminant Wegener's granulomatosis could be a result of enhanced granulocyte differentiation.
ISSN:0028-0836
1476-4687
DOI:10.1038/346520a0