Pathogenesis

PATHOGENESIS

It had been suspected that the genetic defect in thin basement membrane nephropathy would be similar to that in hereditary nephritis (Alport syndrome), since patients with the latter group of disorders also have thin basement membranes early in the course of the disease. This hypothesis has been confirmed in families in which thin basement membrane nephropathy was due to heterozygous defects in COL4A3 or COL4A4, the genes that encode for the alpha-3 and alpha-4 chains of type IV collagen  The entire group of disorders encompassing TBMN and Alport syndrome has been called the Type IV Collagen Nephropathies. - cited from uptodate