Identification of Noncollagenous Sites Encoding Specific Interactions and Quaternary Assembly of alpha 3 alpha 4 alpha 5(IV) Collagen IMPLICATIONS FOR ALPORT GENE THERAPY
Defective assembly of alpha 3 alpha 4 alpha 5(IV) collagen in the glomerular basement membrane causes Alport syndrome, a hereditary glomerulonephritis progressing to end-stage kidney failure. Assembly of collagen IV chains into heterotrimeric molecules and networks is driven by their noncollagenous (NC1) domains, but the sites encoding the specificity of these interactions are not known. To identi