Development of IgA nephropathy-like glomerulonephritis associated with Wiskott-Aldrich syndrome protein deficiency

Détails

ID Serval
serval:BIB_855819419053
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
Development of IgA nephropathy-like glomerulonephritis associated with Wiskott-Aldrich syndrome protein deficiency
Périodique
Clin Immunol
Auteur(s)
Shimizu M., Nikolov N. P., Ueno K., Ohta K., Siegel R. M., Yachie A., Candotti F.
ISSN
1521-7035 (Electronic)
ISSN-L
1521-6616
Statut éditorial
Publié
Date de publication
02/2012
Volume
142
Numéro
2
Pages
160-6
Langue
anglais
Notes
Shimizu, M
Nikolov, N P
Ueno, K
Ohta, K
Siegel, R M
Yachie, A
Candotti, F
eng
Z01 HG000122-10/Intramural NIH HHS/
ZIA HG000122-13/Intramural NIH HHS/
Z99 HG999999/Intramural NIH HHS/
Z01 HG000122-11/Intramural NIH HHS/
ZIA HG000122-14/Intramural NIH HHS/
Comparative Study
Research Support, N.I.H., Intramural
Clin Immunol. 2012 Feb;142(2):160-6. doi: 10.1016/j.clim.2011.10.001. Epub 2011 Oct 19.
Résumé
Wiskott-Aldrich syndrome (WAS) is a rare X-linked disorder caused by mutations in the WAS gene. Glomerulonephritis is a frequent complication, however, histopathological data from affected patients is scarce because the thrombocytopenia that affects most patients is a contraindication to renal biopsies. We found that WASp-deficient mice develop proliferative glomerulonephritis reminiscent of human IgA nephropathy (IgAN). We examined whether increased aberrant IgA production is associated with the development of glomerulonephritis in WASp-deficient mice. Serum IgA and IgA production by splenic B cells was increased in WASp-deficient mice compared to wild-type (WT) mice. A lectin-binding study revealed a reduced ratio of sialylated and galactosylated IgA in the sera from old WASp-deficient mice. Circulating IgA-containing immune complexes showed significantly higher titers in WASp-deficient mice compared to WT mice. These results indicate that the increased IgA production and aberrant glycosylation of IgA may be critically involved in the pathogenesis of glomerulonephritis in WAS.
Mots-clé
Animals, B-Lymphocytes/immunology, Disease Models, Animal, Glomerulonephritis, IGA/*immunology/metabolism/pathology, Glycosylation, Humans, Immunoglobulin A/blood/*metabolism, Mice, Mice, Knockout, Spleen/immunology, Thrombocytopenia/metabolism, Wiskott-Aldrich Syndrome/*immunology, Wiskott-Aldrich Syndrome Protein/*deficiency
Pubmed
Création de la notice
01/11/2017 10:29
Dernière modification de la notice
20/08/2019 14:44
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