Laboratory for Molecular Diagnostics
Center for Nephrology and Metabolic Disorders

Goodpasture syndrome

Goodpasture syndrome is a kidney and lung disease caused by autoantibodies directed against collagen components of the basement membrane. While in the kidney the disease causes progressive renal failure lung bleeding may even cause an emergency. Sometimes the disease is observed in patients with Alport syndrome after successful transplantation.

Diagnosis

The diagnosis is made clinically by the conjunction of kidney (proteinuria, glomerular hematuria and progressive renal failure) and pulmonary (dyspnea and hemoptysis) symptoms. The diagnosis is proved if autoantibodies against glomerular basement membrane are detected.

Pathogenesis

Though Goodpasture syndrome is an acquired disease, it often runs in families, among twins in particular. Association was found with the MHC locus which is not surprising as Goodpasture syndrome is an immunological disorder.

Biochemical studies revealed autoantibodies directed against particular regions of the NC1 domain of the collagen 4 chains 3 and 5, genes COL4A3 and COL4A5 respectively. Alport patients who develop Goodpasture after receiving a renal transplant show a broader spectrum of antibodies.

How a serine/threonine kinase that binds to the same region may be involved in the pathogenesis is yet to be elucidated.

Systematic

Glomerulonephritis
C3 glomerulopathy
CFHR5 Nephropathy
Goodpasture syndrome
COL4A3
COL4A3BP
COL4A5
Lupus erythematosus nephritis
Membranoproliferative glomerulonephritis (MPGN)
Membranous nephropathy
Mesangioproliferative glomerulonephritis

References:

1.

Raya A et al. (1999) Characterization of a novel type of serine/threonine kinase that specifically phosphorylates the human goodpasture antigen.

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2.

Turner N et al. (1992) Molecular cloning of the human Goodpasture antigen demonstrates it to be the alpha 3 chain of type IV collagen.

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3.

Kalluri R et al. (1997) Susceptibility to anti-glomerular basement membrane disease and Goodpasture syndrome is linked to MHC class II genes and the emergence of T cell-mediated immunity in mice.

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4.

Netzer KO et al. (1999) The goodpasture autoantigen. Mapping the major conformational epitope(s) of alpha3(IV) collagen to residues 17-31 and 127-141 of the NC1 domain.

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5.

Hellmark T et al. (1999) Goodpasture disease. Characterization of a single conformational epitope as the target of pathogenic autoantibodies.

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6.

Hudson BG et al. (2003) Alport's syndrome, Goodpasture's syndrome, and type IV collagen.

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7.

Pedchenko V et al. (2010) Molecular architecture of the Goodpasture autoantigen in anti-GBM nephritis.

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8.

Gossain VV et al. (1972) Goodpasture's syndrome: a familial occurrence.

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9.

D'Apice AJ et al. (1978) Goodpasture's syndrome in identical twins.

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10.

Simonsen H et al. (1982) Goodpasture's syndrome in twins.

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11.

Orphanet article

Orphanet ID 375 [^]
12.

OMIM.ORG article

Omim 233450 [^]
13.

Wikipedia article

Wikipedia EN (Goodpasture_syndrome) [^]
Update: April 29, 2019