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2016 ; 172
(ä): 72-77
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IgA nephropathy enigma
#MMPMID27444044
Mestecky J
; Novak J
; Moldoveanu Z
; Raska M
Clin Immunol
2016[Nov]; 172
(ä): 72-77
PMID27444044
show ga
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 IgA1
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.
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