Loss of α3/α4 (IV) collagen from the glomerular basement membrane induces a strain-dependent isoform switch to α5α6 (IV) collagen associated with longer renal …

JS Kang, XP Wang, JH Miner, R Morello… - Journal of the …, 2006 - journals.lww.com
Abstract Mutations in COL4A3/4/5 genes that affect the normal assembly of the α3/4/5 (IV)
collagen network in the glomerular basement membrane (GBM) cause Alport syndrome.
Patients progress to renal failure at variable rates that are determined by the underlying
mutation and putative modifier genes. Col4a3−/− mice, a model for autosomal recessive
Alport syndrome, progress to renal failure significantly slower on the C57BL/6 than on the
129X1/Sv background. Reported here is a novel strain-specific alternative collagen IV …