Alport's syndrome, Goodpasture's syndrome, and type IV collagen

Billy G. Hudson, Karl Tryggvason, Munirathinam Sundaramoorthy, Eric G. Neilson*

*Corresponding author for this work

Research output: Contribution to journalReview article

650 Scopus citations

Abstract

The family of type IV collagens continues to provide an important source of new information about basement-membrane molecules in epithelial tissues. Given the additional knowledge available today, we propose renaming the α3.α4.α5(IV) protomer the "Goodpasture protomer." This change honors the cornerstone role of the Goodpasture antigen in enlarging our knowledge of collagen IV biochemistry and relates the molecular understanding of protomer assembly to the pathogenesis of Goodpasture's and Alport's syndromes. The insights provided by the work completed to date suggest that a number of therapeutic advances may be forthcoming. Recognition that basement membranes in patients with Alport's syndrome are particularly susceptible to proteolysis may eventually lead to the prophylactic use of specific protease inhibitors or even gene-replacement therapy. Work with experimental models of anti-glomerular basement membrane disease already predicts a role for costimulatory blockade of T-cell activation, immune modulation with interleukin-4 and interleukin-10, or inhibition of macrophage migration. If nothing else, the future will be interesting, and work in this area will undoubtedly provide a new understanding of collagen-related diseases. Supported in part by grants (DK-46282 and DK-55926, to Dr. Neilson; DK-18381 and DK-53763, to Dr. Hudson; and DK-62524, to Dr. Sundaramoorthy) from the National Institutes of Health and a grant (to Dr. Tryggvason) from the Swedish Medical Research Council. We are indebted to Larry Howell for assistance in the preparation of the figures.

Original languageEnglish (US)
Pages (from-to)2543-2556
Number of pages14
JournalNew England Journal of Medicine
Volume348
Issue number25
DOIs
StatePublished - Jun 19 2003

ASJC Scopus subject areas

  • Medicine(all)

Fingerprint Dive into the research topics of 'Alport's syndrome, Goodpasture's syndrome, and type IV collagen'. Together they form a unique fingerprint.

  • Cite this