Medizinische Universität Graz - Research portal
Selected Publication:
Jenne, DE; Fröhlich, L; Hummel, AM; Specks, U.
Cloning and functional expression of the murine homologue of proteinase 3: implications for the design of murine models of vasculitis.
FEBS Lett. 1997; 408(2):187-190
Doi: 10.1016/S0014-5793(97)00418-3
Web of Science
PubMed
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- Co-authors Med Uni Graz
- Fröhlich Leopold F.
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- Abstract:
- Anti-neutrophil cytoplasmic autoantibodies recognizing conformational epitopes (c-ANCA) of proteinase 3 (PR3) from azurophil granules are a diagnostic hallmark in Wegener's granulomatosis (WG). Because a functional PR3 homologue has not been identified in rodents, it is difficult to assess immunopathological responses in rats or mice immunized with patients' derived c-ANCA or human PR3. Here we report the full length cDNA cloning and functional expression of murine PR3 in HMC-1 cells. Recombinant murine PR3 shows highly similar substrate specificities towards synthetic peptides and is inhibited by human alpha1-proteinase inhibitor like human PR3. However, neither human c-ANCA, rabbit sera nor mouse monoclonal antibodies to human PR3 recognize the murine homologue. Consequently, it is unlikely that disease observed in mice after immunization with c-ANCA or human PR3 is caused by pathogenic antibodies directed against mouse PR3. Recombinant human-mouse chimaeric variants will be a valuable new tool to localize the disease-specific immunodominant epitopes in human PR3.
- Find related publications in this database (using NLM MeSH Indexing)
- Amino Acid Sequence -
- Animals -
- Antibodies, Antineutrophil Cytoplasmic - immunology
- Antibody Specificity - immunology
- Base Sequence - immunology
- Cell Line - immunology
- Cloning, Molecular - immunology
- Disease Models, Animal - immunology
- Epitopes - chemistry
- Humans - chemistry
- Mast Cells - enzymology
- Mice - enzymology
- Molecular Sequence Data - enzymology
- Myeloblastin - enzymology
- Recombinant Proteins - chemistry
- Sequence Alignment - chemistry
- Serine Endopeptidases - chemistry
- Vasculitis - immunology
- alpha 1-Antitrypsin - pharmacology
- Find related publications in this database (Keywords)
- neutrophil
- proteinase 3
- anti-neutrophil cytoplasmic antibody
- in vivo animal model