Goodpasture's autoimmune disease - A collagen IV disorder.

Published on May 12, 2018in Matrix Biology11.583
· DOI :10.1016/J.MATBIO.2018.05.004
Vadim Pedchenko21
Estimated H-index: 21
(VUMC: Vanderbilt University Medical Center),
A. Richard Kitching57
Estimated H-index: 57
(Monash University),
Billy G. Hudson78
Estimated H-index: 78
(VUMC: Vanderbilt University Medical Center)
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.
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