Digital Ischemia as a Rare Manifestation of C3 Glomerulopathy Associated With Monoclonal Gammopathy
Year: 2023
Type: article
Abstract: C3 glomerulopathy (C3GP) is a group of rare glomerular diseases histologically characterized by isolated or predominant C3 deposits within glomeruli associated with glomerular inflammation. Dysregulation of complement alternative pathway was shown to be the cornerstone of their pathogenesis. Patients usually display either rare and pathogenic variants in complement genes implicated in alternative pathway regulation or, more often, autoantibodies interacting with components of this pathway. Some of these antibodies stabilize the activity of C3 or C5 convertase – C3 and C5 nephritic factors (C3NeF and C5NeF) –, while others inhibit regulatory proteins such as complement factor H (CFH).
Source: Kidney International Reports
Institutions: Hôpital Rothschild, Hôpital Cochin, Fondation Ophtalmologique Adolphe de Rothschild, Fondation de Rothschild, Centre Hospitalier du Mans, Hôpital Européen Georges-Pompidou, Centre Hospitalier Universitaire de Caen Normandie, Centre Hospitalier Universitaire de Toulouse, Centre Hospitalier Universitaire de Montpellier, Hôpital Saint-Louis, Hôpital de la Croix-Rousse
Language: English
Subfield: Immunology
Field: Immunology and Microbiology
Domain: Life Sciences
Open Access status: gold
Estimated APC paid: $2,625
OpenAlex ID: W4389640526