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IgA nephropathy enigma
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SYSNO ASEP 0469665 Document Type J - Journal Article R&D Document Type Journal Article Subsidiary J Článek ve WOS Title IgA nephropathy enigma Author(s) Městecký, Jiří (MBU-M) ORCID
Novák, J. (US)
Moldoveanu, Z. (US)
Raška, M. (CZ)Source Title Clinical Immunology. - : Elsevier - ISSN 1521-6616
Roč. 172, NOV 2016 SI (2016), s. 72-77Number of pages 6 s. Language eng - English Country US - United States Keywords IgA nephropathy ; IgA subclasses ; Autoimmunity Subject RIV EE - Microbiology, Virology R&D Projects NV15-33686A GA MZd - Ministry of Health (MZ) Institutional support MBU-M - RVO:61388971 UT WOS 000388056200012 EID SCOPUS 84993999910 DOI 10.1016/j.clim.2016.07.011 Annotation IgA nephropathy (IgAN) is the leading cause of primary glomerulonephritis in the world. The disease is characterized by the presence of IgA-containing immune complexes in the circulation and in mesangial deposits with ensuing glomerular injury. Although in humans there are two IgA subclasses, only IgA1 molecules are involved. The exclusivity of participation of IgA1 in IgAN prompted extensive structural and immunological studies of the unique hinge region (HR) of IgA1, which is absent in otherwise highly homologous IgA2. HR of IgA1 with altered O-glycans serves as an antigen recognized by autoantibodies specific for aberrant HR glycans leading to the generation of nephritogenic immune complexes. However, there are several unresolved questions concerning the phylogenetic origin of human IgA1 HR, the structural basis of its antigenicity, the origin of antibodies specific for HR with altered glycan moieties, the regulatory defects in IgAl glycosylation pathways, and the potential approaches applicable to the disease-specific interventions in the formation of nephritogenic immune complexes. This review focuses on the gaps in our knowledge of molecular and cellular events that are involved in the immunopathogenesis of IgAN. Workplace Institute of Microbiology Contact Eliška Spurná, eliska.spurna@biomed.cas.cz, Tel.: 241 062 231 Year of Publishing 2017
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