Goodpasture's autoimmune disease - A collagen IV disorder

Matrix Biology : Journal of the International Society for Matrix Biology
Vadim PedchenkoBilly G Hudson

Abstract

Goodpasture's (GP) disease is an autoimmune disorder characterized by the deposition of pathogenic autoantibodies in basement membranes of kidney and lung eliciting rapidly progressive glomerulonephritis and pulmonary hemorrhage. The principal autoantigen is the α345 network of collagen IV, which expression is restricted to target tissues. Recent discoveries include a key role of chloride and bromide for network assembly, a novel posttranslational modification of the antigen, a sulfilimine bond that crosslinks the antigen, and the mechanistic role of HLA in genetic susceptibility and resistance to GP disease. These advances provide further insights into molecular mechanisms of initiation and progression of GP disease and serve as a basis for developing of novel diagnostic tools and therapies for treatment of Goodpasture's disease.

Citations

Apr 12, 2019·The FEBS Journal·Alberto PassiRenato V Iozzo
Jan 6, 2019·Pharmaceuticals·Joana NevesMartina U Muckenthaler
Dec 29, 2020·Frontiers in Neurology·Austin NegronThomas G Forsthuber
Jan 21, 2021·Bioengineering·Yujia Xu, Michele Kirchner

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