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Literature summary for 3.4.21.76 extracted from

  • Hua, F.; Wilde, B.; Dolff, S.; Witzke, O.
    T-lymphocytes and disease mechanisms in Wegeners granulomatosis (2009), Kidney Blood Press. Res., 32, 389-398.
    View publication on PubMed
Search for more literature for 3.4.21.76

Cloned(Commentary)

Cloned (Comment) Organism
mice immunized with recombinant or synthetic human complementary PR3 Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
cell surface neutrophil Homo sapiens 9986
-

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
leukocyte
-
 Homo sapiens
-
additional information PR3 antigen is usually present in granulomas and antigen-presenting cells are found in these lesions as well Homo sapiens
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neutrophil
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 Homo sapiens
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serum presence of anti-complementary PR3 and anti-PR3 antibodies in human Wegener’s granulomatosis sera Homo sapiens
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Synonyms

Synonyms Comment Organism
PR-3
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 Homo sapiens
PR3
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 Homo sapiens
Proteinase-3
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 Homo sapiens

General Information

General Information Comment Organism
physiological function occurrence of anti-neutrophil-cytoplasmic antibodies (ANCA) directed against proteinase-3 is a hallmark of Wegener’s granulomatosis. Association of anti-PR3 titers and disease activity, which remains controversial. PR3 antigen elicits strong Th1-responses via dendritic cell maturation and subsequent antigen presentation to T-cells. Initial immune response in autoimmunity is directed against complementary PR3, resulting in the formation of antibodies against complementary PR3 (idiotypic response). Later on, an anti-idiotypic response against anti-complementary PR3 antibodies evolves. The antibodies formed during this anti-idiotypic response react to the sense autoantigen PR3. ANCA produced by granuloma-resident B cells bind to primed neutrophils that express PR3 on their surface. Genetic sequences complementary to human PR3 gene are detected in pathogens like Staphylococcus aureus, Ross River virus and Entamoeba histolytica. complementary PR3 transcripts are absent from healthy controls, PR3-ANCA-negative or lupus patients. Complementary PR3 mRNA is present in leukocytes of PR3-ANCA patients Homo sapiens