Anti-GBM antibodies in Goodpasture syndrome; anatomy of an epitope.

like group (comprising a-2(IV ) itself, a-4(IV ), and Introduction a-6(IV )). Basement membranes are thin sheet-like extracellular The six a(IV ) chains di er considerably from each structures that form an anatomical barrier wherever other in their tissue distribution. The a-1( IV ) and cells meet connective tissue. They provide a substrate a-2(IV ) chains are found in all basement membranes, for organs and cells and relay signals important for whereas the a-3( IV ) and a-4( IV ) chains are the di erentiation and maintenance of the tissue. The co-localized in some specialized membranes, for importance of a functional basement membrane is example the GBM, lung, lens capsule, cochlea, brain, illustrated in several pathological conditions where the and testis [3]. The a-5(IV ) and a-6(IV ) chains also glomerular basement membrane (GBM) is disrupted. have a limited tissue distribution and are found, for An example is Goodpasture syndrome, where an autoexample, in skin, lens capsule, smooth muscle and immune reaction to the GBM rapidly causes life kidney. The a-5( IV) and a-6(IV ) chains also seem to threatening disease and loss of kidney function. be co-localized, except in the GBM which appears to