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Matrix Biol. 2018 Oct;71-72:240-249. doi: 10.1016/j.matbio.2018.05.004. Epub 2018 May 12.

Goodpasture's autoimmune disease - A collagen IV disorder.

Author information

1
Department of Medicine, Division of Nephrology and Hypertension, Vanderbilt University Medical Center, Nashville, United States; Center for Matrix Biology, Department of Biochemistry, Department of Pathology, Microbiology and Immunology, Department of Cell and Developmental Biology, Vanderbilt Ingram Cancer Center, Vanderbilt Institute of Chemical Biology, Vanderbilt University Medical Center, Nashville, TN, United States. Electronic address: vadim.pedchenko@vanderbilt.edu.
2
Centre for inflammatory diseases, Monash University Department of Medicine, 246 Clayton Rd, Clayton, VIC 3168, Australia; Department of Nephrology, Monash Health, 246 Clayton Rd, Clayton, VIC 3168, Australia; Department and Pediatric Nephrology, Monash Health, 246 Clayton Rd, Clayton, VIC 3168, Australia.
3
Department of Medicine, Division of Nephrology and Hypertension, Vanderbilt University Medical Center, Nashville, United States; Center for Matrix Biology, Department of Biochemistry, Department of Pathology, Microbiology and Immunology, Department of Cell and Developmental Biology, Vanderbilt Ingram Cancer Center, Vanderbilt Institute of Chemical Biology, Vanderbilt University Medical Center, Nashville, TN, United States.

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.

PMID:
29763670
PMCID:
PMC6482832
DOI:
10.1016/j.matbio.2018.05.004
[Indexed for MEDLINE]
Free PMC Article

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